General Information of Disease (ID: DISQPLA4)

Disease Name Huntington disease
Synonyms Huntington's Disease; Huntington's disease; HD; Huntington's chorea; Huntington chorea; Huntington disease
Disease Class 8A01: Choreiform disorder
Definition Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia.
Disease Hierarchy
DISYMF71: Huntington disease and related disorders
DISOJJ2D: Movement disorder
DISQPLA4: Huntington disease
ICD Code
ICD-11
ICD-11: 8A01.10
ICD-10
ICD-10: G10
ICD-9
ICD-9: 294.1, 333.4
Expand ICD-9
294.1,333.4
Disease Identifiers
MONDO ID
MONDO_0007739
MESH ID
D006816
UMLS CUI
C0020179
OMIM ID
143100
MedGen ID
5654
Orphanet ID
399
SNOMED CT ID
58756001

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 2 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Deutetrabenazine DMUPFLI Approved Small molecular drug [1]
Tetrabenazine DMYWQ0O Approved Small molecular drug [2]
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This Disease is Treated as An Indication in 20 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
IONIS-HTTRX DM4KH1L Phase 3 NA [3]
Laquinimod DM3IWS8 Phase 3 Small molecular drug [4]
Pridopidine DMX9YLR Phase 3 Small molecular drug [5]
Tominersen DM7ERXY Phase 3 NA [6]
RP103 DM5FHUP Phase 2/3 NA [7]
Coenzyme Q10 analog DMO0VGH Phase 2 NA [8]
OMS824 DMA4OGL Phase 2 NA [9]
PBT-2 DMKWGS1 Phase 2 Small molecular drug [7]
PF-02545920 DMJPE61 Phase 2 Small molecular drug [7]
SEN-196 DMLDBQ5 Phase 2 Small molecular drug [10]
SOM3355 DMKCBSM Phase 2 NA [11]
SRX246 DMKOTUJ Phase 2 Small molecular drug [12]
VX-15 DMLWM4G Phase 2 Antibody [4]
AMT-130 DM20B90 Phase 1/2 Gene therapy [13]
SBT-20 DM8EI0J Phase 1/2 NA [4]
WVE-120101 DMYDCW4 Phase 1/2 NA [4]
WVE-120102 DMMDP71 Phase 1/2 NA [4]
123I-MNI-420 DMVCF7L Phase 1 NA [14]
GSK-356278 DMIEAMU Phase 1 Small molecular drug [15]
PBF-999 DMBSQH9 Phase 1 Small molecular drug [16]
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⏷ Show the Full List of 20 Drug(s)
This Disease is Treated as An Indication in 3 Preclinical Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
ALN-HTT DM8T6H7 Preclinical RNAi therapeutics [17]
CHDI-340246 DMYGITU Preclinical Small molecular drug [18]
Huntingtin gene antisense DM3NP4I Preclinical Antisense drug [19]
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This Disease is Treated as An Indication in 11 Investigative Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
AC-0523 DMJ1WML Investigative NA [20]
AMT-090 DMB0D2Y Investigative NA [20]
Calmidazolium DM5ZTJL Investigative Small molecular drug [21]
CHDI-00316226 DM3BDXI Investigative NA [20]
CHDI-003940246 DM257AG Investigative NA [20]
Gene therapy, Huntington's disease DMV4NYM Investigative NA [20]
ISIS-387898 DMB9DT1 Investigative NA [20]
OG-Cx-833 DM29WXM Investigative NA [20]
PRO-289 DMPM8V0 Investigative NA [20]
QBP1 mimetics DMKQR1V Investigative NA [20]
ReN-005 DMC3B2H Investigative NA [20]
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⏷ Show the Full List of 11 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 203 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
ACE2 TTUI5H7 Limited Genetic Variation [22]
ADCYAP1 TTW4LYC Limited Altered Expression [23]
CASP2 TT12VNG Limited Biomarker [24]
GAL TTXZAJ5 Limited Biomarker [25]
GAP43 TTSGLN5 Limited Biomarker [25]
GCG TT6Y4PN Limited Biomarker [26]
MAOA TT3WG5C Limited Biomarker [27]
NEK1 TTO5QT2 Limited Genetic Variation [28]
PTPN13 TT405FP Limited Biomarker [29]
SLC29A1 TTLXAKE Limited Biomarker [30]
TNNI3 TTNLDK6 Limited Biomarker [31]
CREB1 TTH4AN3 moderate Altered Expression [32]
DIABLO TTN74LE moderate Biomarker [33]
EIF2AK2 TTXEZJ4 moderate Altered Expression [34]
GDNF TTF23ML moderate Altered Expression [35]
LEP TTBJEZ5 moderate Biomarker [36]
MAOB TTGP7BY moderate Biomarker [37]
NPY TT64REZ moderate Biomarker [38]
SOST TTYRO4F moderate Altered Expression [39]
TREM2 TTQRMSJ moderate Biomarker [40]
TRPC5 TT32NQ1 moderate Biomarker [41]
UBE3A TTUZX6V moderate Biomarker [42]
AADAT TTT3IXG Strong Altered Expression [43]
ABAT TTT2LD9 Strong Therapeutic [44]
ACO2 TTMTF2P Strong Altered Expression [45]
ACVR2B TTLFRKS Strong Biomarker [46]
ADORA2A TTM2AOE Strong Altered Expression [47]
ADORA2B TTNE7KG Strong Genetic Variation [48]
AHSG TTKF4WV Strong Altered Expression [49]
ALPI TTHYMUV Strong Altered Expression [50]
AOC3 TT7HC21 Strong Biomarker [51]
APC2 TTSMK36 Strong Biomarker [52]
APOE TTKS9CB Strong Genetic Variation [53]
AQP9 TTQEI32 Strong Biomarker [54]
AR TTKPW01 Strong Biomarker [55]
ASIC1 TTRJYB6 Strong Biomarker [56]
ATG7 TTLVB9Z Strong Biomarker [57]
ATP2A2 TTE6THL Strong Biomarker [58]
BAX TTQ57WJ Strong Therapeutic [59]
C5 TTKANGO Strong Altered Expression [60]
CACNA1A TTX4QDJ Strong Genetic Variation [61]
CASP6 TTKW4ML Strong Biomarker [62]
CBS TTVZJ7G Strong Biomarker [63]
CD200 TT0BE68 Strong Altered Expression [64]
CDK5 TTL4Q97 Strong Biomarker [65]
CETP TTFQAYR Strong Genetic Variation [66]
CHAT TTKYFSB Strong Biomarker [67]
CHRM1 TTZ9SOR Strong Biomarker [68]
CLCN1 TTUYAF3 Strong Biomarker [69]
CNR1 TT6OEDT Strong Biomarker [70]
CNR2 TTMSFAW Strong Biomarker [71]
CNTN1 TTPR8FK Strong Biomarker [72]
CREBBP TTFRCTK Strong Altered Expression [73]
CRTC1 TT4GO0F Strong Biomarker [74]
CS TTZA6B3 Strong Altered Expression [75]
CTDSP1 TTHZAF0 Strong Biomarker [76]
CYP46A1 TT4EB85 Strong Biomarker [77]
DCLK3 TTMYK4Z Strong Biomarker [78]
DDIT4 TTVEOY6 Strong Altered Expression [79]
DLG4 TT9PB26 Strong Biomarker [80]
DNAJB1 TTPXAWS Strong Altered Expression [81]
DPYSL2 TTZCW3T Strong Biomarker [82]
DRD1 TTZFYLI Strong Biomarker [83]
DRD2 TTEX248 Strong Biomarker [84]
DRD3 TT4C8EA Strong Biomarker [85]
DRD5 TTS2PH3 Strong Biomarker [86]
DUSP1 TTG8HIM Strong Therapeutic [87]
E2F1 TTASI04 Strong Biomarker [88]
E2F2 TT5FYX0 Strong Genetic Variation [89]
EIF4E TTZGCP6 Strong Altered Expression [73]
EPHA3 TTHS2LR Strong Biomarker [90]
EZH1 TTNJA0C Strong Biomarker [91]
F11 TTDM4ZU Strong Biomarker [92]
F13A1 TTXI2RA Strong Altered Expression [93]
F13B TTAXGIP Strong Altered Expression [93]
FAP TTGPQ0F Strong Biomarker [94]
FGF23 TT2IZ4K Strong Altered Expression [95]
FUS TTKGYZ9 Strong Biomarker [96]
GABRA2 TTBMV1G Strong Genetic Variation [97]
GAD2 TT7UY6K Strong Biomarker [98]
GFAP TTI6FFX Strong Biomarker [99]
GIP TT40HS5 Strong Biomarker [100]
GLO1 TTV9A7R Strong Genetic Variation [101]
GPR3 TTHZVSK Strong Biomarker [102]
GPRC5D TTHRAPJ Strong Biomarker [103]
GRIN1 TTLD29N Strong Biomarker [104]
GRIN2A TTKJEMQ Strong Biomarker [105]
GRIN2B TTN9D8E Strong Altered Expression [106]
GRM5 TTHS256 Strong Biomarker [107]
GSR TTEP6RV Strong Therapeutic [108]
HAAO TTWON83 Strong Biomarker [109]
HAMP TTRV5YJ Strong Altered Expression [110]
HCRT TTU5HJP Strong Biomarker [111]
HDAC1 TT6R7JZ Strong Altered Expression [112]
HDAC3 TT4YWTO Strong Biomarker [113]
HDAC4 TTTQGH8 Strong Biomarker [114]
HDAC8 TTT6LFV Strong Biomarker [115]
HSF1 TTN6STZ Strong Biomarker [116]
HTR1A TTSQIFT Strong Biomarker [117]
IAPP TTHN8EM Strong Biomarker [118]
IDUA TT0IUKX Strong Genetic Variation [119]
IFNL3 TTRF4Q2 Strong Biomarker [120]
IL12A TTRTK6Y Strong Genetic Variation [121]
IL12B TTGW72V Strong Genetic Variation [122]
ITPR1 TT5HWAT Strong Biomarker [123]
ITPR3 TTH1769 Strong Biomarker [124]
JAG2 TTOJY1B Strong Biomarker [102]
KDM5C TT94UCF Strong Biomarker [125]
KMO TTIY56R Strong Biomarker [126]
LAMC2 TTNS7H3 Strong Biomarker [127]
LIMK1 TTWL9TY Strong Biomarker [128]
LTB TTHQ6US Strong Biomarker [129]
MAP2K2 TT8H9GB Strong Genetic Variation [130]
MAP3K1 TTW8TJI Strong Genetic Variation [131]
MAPK10 TT056SO Strong Altered Expression [132]
MAPK11 TT73U6C Strong Altered Expression [133]
MBP TT2RY5P Strong Biomarker [134]
MC1R TT0MV2T Strong Genetic Variation [135]
MSTN TTM8I2X Strong Biomarker [136]
MTDH TTH6SA5 Strong Biomarker [137]
MTNR1A TT0WAIE Strong Altered Expression [138]
NAAA TTMN4HY Strong Biomarker [139]
NFE2L2 TTA6ZN2 Strong Biomarker [140]
NGFR TTEDJN4 Strong Biomarker [141]
NPEPPS TT371QC Strong Altered Expression [142]
NPY2R TTJ6WK9 Strong Biomarker [38]
NPY5R TTY6EWA Strong Biomarker [143]
NR1H3 TTECBXN Strong Genetic Variation [144]
NTF4 TTIM2WO Strong Biomarker [145]
NTRK2 TTKN7QR Strong Biomarker [146]
OGG1 TTRU01G Strong Genetic Variation [147]
PAM TTF4ZPC Strong Genetic Variation [148]
PDE10A TTJW4LU Strong Biomarker [149]
PDE1B TT3ZS42 Strong Altered Expression [150]
PDE4A TTZ97H5 Strong Biomarker [151]
PDK3 TTDEQIP Strong Altered Expression [152]
PGD TTZ3IFB Strong Biomarker [153]
PHGDH TT8DRCK Strong Biomarker [74]
PIN1 TTJNTSI Strong Altered Expression [154]
PKN2 TTTHO0M Strong Biomarker [128]
PNKP TTHR3IE Strong Altered Expression [155]
PNP TTMCF1Y Strong Biomarker [156]
PPARD TT2JWF6 Strong Biomarker [157]
PPARGC1B TTKSQ3W Strong Altered Expression [158]
PPT1 TTSQC14 Strong Biomarker [159]
PTH TT6F7GZ Strong Biomarker [160]
QPCT TTJ7YTV Strong Biomarker [161]
REG3A TTL4H8N Strong Biomarker [162]
RGS12 TTP9AV7 Strong Genetic Variation [163]
RGS2 TTKB7T3 Strong Biomarker [164]
RIPK2 TTCQ2E5 Strong Genetic Variation [165]
RPH3A TT9L4J8 Strong Biomarker [166]
RPS6KA1 TTIXKA4 Strong Altered Expression [167]
RXRA TT6PEUO Strong Genetic Variation [144]
S100A12 TTQ4ESF Strong Biomarker [168]
S1PR5 TTDZCKV Strong Biomarker [169]
SCN4A TT84DRB Strong Genetic Variation [170]
SGCA TTS9Q5V Strong Biomarker [171]
SGPL1 TT618Q2 Strong Biomarker [172]
SIGMAR1 TT5TPI6 Strong Biomarker [173]
SIRT2 TTLKF5M Strong Biomarker [174]
SIRT3 TTVZLIJ Strong Altered Expression [175]
SLC11A2 TT2IS7P Strong Altered Expression [176]
SLC12A1 TTS087L Strong Biomarker [177]
SLC12A5 TTH6UZY Strong Biomarker [178]
SLC18A2 TTNZRI3 Strong Genetic Variation [179]
SLC1A1 TTG2A6F Strong Altered Expression [180]
SLC1A2 TT2F078 Strong Biomarker [181]
SLC1A3 TT8WRDA Strong Biomarker [182]
SLC1A6 TT6KMPN Strong Biomarker [182]
SLC40A1 TT6Y1PG Strong Altered Expression [183]
SLC6A4 TT3ROYC Strong Genetic Variation [184]
SLCO1B3 TTU86P0 Strong Genetic Variation [185]
SNCA TT08OSU Strong Biomarker [186]
SNCG TT5TQNZ Strong Biomarker [187]
SORT1 TTRX9AV Strong Biomarker [188]
SPHK2 TTCN0M9 Strong Biomarker [189]
SPN TTOZAX0 Strong Biomarker [190]
ST8SIA4 TTDP8YM Strong Genetic Variation [191]
SV2A TTT3P91 Strong Altered Expression [192]
TARDBP TT9RZ03 Strong Biomarker [186]
TGM1 TT7A949 Strong Altered Expression [193]
TNFRSF17 TTZ3P4W Strong Biomarker [194]
TRIP10 TTKHTGE Strong Biomarker [195]
TRPC1 TTA76X0 Strong Altered Expression [196]
TRPC4 TTX0H5W Strong Biomarker [197]
TUSC2 TTJ8O14 Strong Biomarker [162]
UBC TTBP3XA Strong Genetic Variation [198]
UCHL1 TTX9IFP Strong Genetic Variation [199]
UCP1 TTI12YJ Strong Biomarker [200]
ULK1 TT4D7MJ Strong Altered Expression [107]
UTRN TTNO1VA Strong Biomarker [201]
VCP TTHNLSB Strong Biomarker [202]
VIPR2 TT4O5P0 Strong Altered Expression [203]
WDR5 TT7OFWB Strong Altered Expression [204]
WWP1 TTBWMKT Strong Biomarker [205]
ADCYAP1R1 TT5OREU Definitive Biomarker [23]
HTT TTIWZ0O Definitive Autosomal dominant [206]
KCNA1 TTS3DIK Definitive Genetic Variation [207]
KCNA5 TTW0CMT Definitive Genetic Variation [207]
KCNA6 TTJ2W69 Definitive Genetic Variation [207]
NPSR1 TTV1C0Z Definitive Genetic Variation [208]
RAB7A TTF6WAQ Definitive Biomarker [209]
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⏷ Show the Full List of 203 DTT(s)
This Disease Is Related to 6 DTP Molecule(s)
Gene Name DTP ID Evidence Level Mode of Inheritance REF
ABCD1 DTKM9DZ Strong Biomarker [210]
SLC12A2 DTHKL3Q Strong Biomarker [178]
SLC25A28 DT4NW1F Strong Altered Expression [211]
SLC2A3 DT9SQ3L Strong Altered Expression [212]
SLC39A8 DTLPQGT Strong Biomarker [176]
SLC9A5 DTI6LSR Strong Biomarker [213]
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⏷ Show the Full List of 6 DTP(s)
This Disease Is Related to 13 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
FAAH DEUM1EX Limited Altered Expression [214]
NAT10 DEZV4AP Disputed Biomarker [215]
ACP3 DEDW5H6 Strong Biomarker [162]
CRMP1 DE0EUXB Strong Biomarker [201]
FXN DEXVHDB Strong Altered Expression [211]
GPX6 DEOS3FD Strong Altered Expression [216]
GSTO2 DEHMPZR Strong Genetic Variation [217]
IPMK DESA9EX Strong Biomarker [218]
MT1A DE5ME8A Strong Altered Expression [138]
NT5C2 DE1DOKJ Strong Biomarker [219]
PSAT1 DEBS17P Strong Biomarker [220]
SCLY DEH4TD6 Strong Genetic Variation [221]
TGM3 DEOEB3Q Strong Genetic Variation [222]
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⏷ Show the Full List of 13 DME(s)
This Disease Is Related to 280 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
AIFM1 OTKPWB7Q Limited Biomarker [223]
APAF1 OTJWIVY0 Limited Altered Expression [224]
ATXN1 OTQF0HNR Limited Biomarker [225]
CCHCR1 OT22C116 Limited Biomarker [226]
CFAP54 OTS4SW4H Limited Genetic Variation [28]
CUL1 OTXPE1UZ Limited Altered Expression [227]
ERFE OTSES1HA Limited Biomarker [228]
FGF9 OT2SKDGM Limited Biomarker [229]
LZTS3 OTFFMOJN Limited Genetic Variation [28]
MTHFSD OTB5ZUYF Limited Genetic Variation [28]
NRSN1 OT1KKXC8 Limited Biomarker [25]
PRPF40A OT6EXJZN Limited Biomarker [230]
RELN OTLKMW1O Limited Biomarker [231]
RNF4 OTCMXQRE Limited Altered Expression [232]
SELENBP1 OT3NZNTR Limited Biomarker [226]
SKP1 OT5BPAZ4 Limited Biomarker [227]
TBP OT6C0S52 Limited Biomarker [225]
TCERG1 OTH1LCGH Limited Biomarker [233]
ATRNL1 OTY5JUX2 Disputed Biomarker [215]
PDLIM3 OTVXQC81 Disputed Biomarker [215]
ALPP OTZU4G9W moderate Biomarker [215]
CLDN5 OTUX60YO moderate Altered Expression [234]
IP6K2 OTCUMUUP moderate Biomarker [235]
MGP OTZWU3FU moderate Biomarker [236]
ABCB10 OT1C44F9 Strong Biomarker [237]
ACBD3 OTMYNY7A Strong Biomarker [238]
ACO1 OT2VUR7L Strong Altered Expression [239]
ACSBG1 OTM040MW Strong Biomarker [240]
ADD1 OTTF68DC Strong Genetic Variation [163]
ADGB OT1TM9DT Strong Genetic Variation [241]
AGO2 OT4JY32Q Strong Genetic Variation [242]
AKAP8L OTM3ZHGU Strong Biomarker [243]
ALDH7A1 OTV57BZD Strong Biomarker [244]
ANXA3 OTDD8OI7 Strong Biomarker [54]
ARFGEF2 OTM3CQZT Strong Altered Expression [245]
ARFIP2 OTK9JXIJ Strong Altered Expression [246]
ASAP1 OT4DLRYY Strong Biomarker [162]
ASAP2 OTGEXULW Strong Biomarker [162]
ATAD3A OTWF6HBP Strong Biomarker [201]
ATF5 OT03QCLM Strong Biomarker [247]
ATF6 OTAFHAVI Strong Biomarker [248]
ATF7IP OTU6ZA7F Strong Biomarker [249]
ATXN7 OTL3YF1H Strong Biomarker [250]
BCL11B OT8KKCVJ Strong Biomarker [251]
BLZF1 OT5QD8FG Strong Biomarker [252]
BTRC OT2EZDGR Strong Biomarker [253]
CALB1 OTM7IXDG Strong Biomarker [254]
CALB2 OTSNMCG9 Strong Biomarker [67]
CALD1 OTNJKJ6Q Strong Genetic Variation [255]
CAMK2A OTJGX19T Strong Biomarker [256]
CARD16 OT4NUHWB Strong Genetic Variation [165]
CAST OTBXZZGF Strong Biomarker [257]
CCL4L2 OTDBSXOU Strong Altered Expression [258]
CCT2 OTW1VV4E Strong Genetic Variation [259]
CD200R1 OT65Q9M6 Strong Altered Expression [64]
CERS1 OT6EYRM3 Strong Altered Expression [260]
CFAP97 OT0RSQO4 Strong Altered Expression [261]
CHCHD2 OTL5PA3Y Strong Biomarker [262]
CHI3L1 OT2Z7VJH Strong Biomarker [263]
CHRDL2 OTJU2I5H Strong Biomarker [264]
CKB OTUCKOTT Strong Altered Expression [265]
CMTR2 OT82FBWS Strong Biomarker [266]
COIL OTP4I4DL Strong Biomarker [267]
COL4A2 OTJK1LKN Strong Biomarker [268]
CPLX2 OTU5QWFH Strong Biomarker [269]
CPQ OTTNZNLD Strong Altered Expression [270]
CSH1 OT33HTRR Strong Biomarker [271]
CSH2 OTW8JVAN Strong Biomarker [271]
CUX1 OTU1LCNJ Strong Biomarker [272]
CXCL3 OTSL94KH Strong Altered Expression [273]
CYSTM1 OT1GYXDM Strong Biomarker [54]
DAB2 OTRMQTMZ Strong Altered Expression [274]
DAPK2 OTWODUQG Strong Biomarker [201]
DAXX OTX6O7PL Strong Biomarker [275]
DCT OTYVNTBG Strong Biomarker [197]
DENR OTXP9HOY Strong Biomarker [201]
DGKE OTWS86AS Strong Altered Expression [276]
DISC1 OT43AW4H Strong Altered Expression [277]
DLG1 OTCRZYWT Strong Biomarker [278]
DNAH6 OTUZNWKV Strong Altered Expression [279]
DNAJA1 OT38BZQQ Strong Biomarker [280]
DNAJA2 OTKF1VBT Strong Biomarker [281]
DNAJB2 OTZHPV5M Strong Altered Expression [193]
DNAJB6 OTMHIIAN Strong Altered Expression [282]
DNAJC14 OT7DPIVN Strong Biomarker [281]
DNM1L OTXK1Q1G Strong Biomarker [201]
DPP6 OTWW3H0K Strong Genetic Variation [283]
DROSHA OTCE68KZ Strong Genetic Variation [284]
DTX1 OTYX91DX Strong Genetic Variation [285]
ELK1 OTH9MXD6 Strong Biomarker [286]
EMP1 OTSZHUHQ Strong Biomarker [287]
ENHO OT91QASK Strong Genetic Variation [144]
ERCC6 OT2QZKSF Strong Biomarker [271]
EVA1A OTCY3Q2M Strong Genetic Variation [288]
F8A1 OTXTQ59R Strong Biomarker [289]
FAM193A OTT0W53G Strong Biomarker [290]
FAN1 OT1LM1HZ Strong Genetic Variation [291]
FEN1 OT6QGG7O Strong Biomarker [292]
FIS1 OT2HL10J Strong Biomarker [293]
FLVCR1 OT9XCFOC Strong Biomarker [294]
FOXO4 OT90X9LN Strong Altered Expression [295]
GABBR2 OT67RIFY Strong Genetic Variation [97]
GABPA OT9YB2SA Strong Biomarker [140]
GBA2 OTOZXG5D Strong Genetic Variation [296]
GIT1 OTHO92S5 Strong Biomarker [297]
GPR52 OTPR99C7 Strong Genetic Variation [103]
GPRC5C OT45AJT3 Strong Biomarker [103]
GPX7 OTINT9Z4 Strong Genetic Variation [216]
GRIN3A OTQS9GYY Strong Biomarker [298]
GRINA OTMC1O66 Strong Biomarker [104]
GRK4 OTUU8V2S Strong Genetic Variation [163]
GYS1 OTJZJRWK Strong Altered Expression [299]
H2AX OT18UX57 Strong Biomarker [300]
HACD1 OTEC7EP7 Strong Biomarker [301]
HACE1 OTEZULKD Strong Biomarker [302]
HAND1 OTN4IPVV Strong Altered Expression [274]
HAP1 OT6SG0JQ Strong Biomarker [42]
HCLS1 OTX7WGYN Strong Biomarker [272]
HES4 OTCGMQDT Strong Biomarker [102]
HIP1 OT7AKCFQ Strong Biomarker [303]
HIPK3 OT4WYQM2 Strong Biomarker [275]
HJV OT4235J2 Strong Altered Expression [304]
HOMER1 OTWFD3SI Strong Biomarker [254]
HPCA OTERUPP6 Strong Altered Expression [305]
HSD17B6 OTSB55D2 Strong Biomarker [306]
HSPA4 OT5HR0AR Strong Biomarker [307]
HTRA2 OTC7616F Strong Biomarker [308]
HYPK OTRWRVWN Strong Biomarker [309]
IER3 OTZJI5FZ Strong Biomarker [310]
IFT57 OTXYC20V Strong Biomarker [311]
IGFALS OTTWCZYM Strong Biomarker [312]
IL17RA OTVVI8ER Strong Altered Expression [313]
IL34 OTZ15VVK Strong Biomarker [314]
IMMT OTBDSLE7 Strong Altered Expression [315]
KALRN OT8WRCBH Strong Biomarker [316]
KCNH4 OTHJ8WTU Strong Biomarker [286]
KCNH8 OT3I5FLB Strong Biomarker [286]
KCNIP3 OTCQPEM4 Strong Biomarker [317]
KDM5D OTEKG0KD Strong Altered Expression [318]
KHSRP OTDHZARB Strong Biomarker [272]
KPNA1 OTEMVAJ6 Strong Genetic Variation [319]
KRT7 OTLT3JFN Strong Genetic Variation [221]
LIN7A OTFL3PUX Strong Altered Expression [72]
LIN7B OTX1BC5B Strong Biomarker [72]
LY6E OTMG16BZ Strong Biomarker [320]
MACROH2A1 OTV2DQDD Strong Biomarker [321]
MAP1S OT5WUD4C Strong Biomarker [322]
MAP2 OT6UYT3X Strong Altered Expression [261]
MAP2K4 OTZPZX11 Strong Altered Expression [323]
MAP2K6 OTK13JKC Strong Altered Expression [130]
MARCHF8 OTH7PNN2 Strong Biomarker [324]
MCIDAS OTK1JVAH Strong Biomarker [325]
METRN OTI2645G Strong Biomarker [326]
MLH3 OT91PPBI Strong Genetic Variation [327]
MLXIP OT30UNI7 Strong Biomarker [324]
MNAT1 OTXLOYCB Strong Biomarker [328]
MOK OTQK7M9V Strong Altered Expression [329]
MRPS30 OTDXIAGG Strong Biomarker [162]
MSANTD1 OT4NKG1S Strong Genetic Variation [163]
MSH3 OTD3YPVL Strong Genetic Variation [330]
MSN OTZJ4J6G Strong Biomarker [331]
MSX1 OT5U41ZP Strong Genetic Variation [332]
MT1B OTUA4FFH Strong Altered Expression [138]
MT1E OTXJKU4Y Strong Altered Expression [138]
MT1F OTZVUYG1 Strong Altered Expression [138]
MT1G OTAV1OCR Strong Altered Expression [138]
MT1H OT0MVBM6 Strong Altered Expression [138]
MT1M OTVT8PLU Strong Altered Expression [138]
MT1X OT9AKFVS Strong Altered Expression [138]
MTPAP OT6HQ02S Strong Biomarker [162]
MYLIP OTL0PFGV Strong Biomarker [324]
NAIP OTLA925F Strong Biomarker [305]
NCKIPSD OT24UORN Strong Genetic Variation [333]
ND3 OT1OC3K3 Strong Biomarker [334]
NDUFA1 OTKBUQXP Strong Biomarker [335]
NEFH OTMSCW5I Strong Altered Expression [336]
NEFL OTQESJV4 Strong Biomarker [337]
NEUROD1 OTZQ7QJ2 Strong Biomarker [338]
NFATC4 OTTDCUAO Strong Biomarker [339]
NGB OTW0SIUY Strong Biomarker [340]
NGDN OTBXTCN5 Strong Biomarker [341]
NNAT OTNRLO7G Strong Altered Expression [342]
NR5A1 OTOULYR4 Strong Genetic Variation [343]
NREP OT2AZPKK Strong Genetic Variation [344]
NRGN OTVGE10W Strong Biomarker [345]
NSG1 OTRIMA50 Strong Genetic Variation [346]
NUB1 OTWA6RTX Strong Biomarker [347]
OPN1LW OTFNUZ7O Strong Altered Expression [73]
OPN4 OT1LZ7TS Strong Altered Expression [348]
OPTN OT2UXWH9 Strong Biomarker [215]
OR10A4 OTYYB8SY Strong Altered Expression [349]
OSR1 OTB19LEQ Strong Altered Expression [274]
P4HB OTTYNYPF Strong Altered Expression [350]
PACSIN1 OT07Z2RQ Strong Biomarker [351]
PAG1 OTFOJUIQ Strong Altered Expression [73]
PANK2 OTFBW889 Strong Biomarker [352]
PAPOLA OTPHD65D Strong Biomarker [162]
PAPPA2 OTXYGTUZ Strong Biomarker [353]
PCBP1 OTHN0TD7 Strong Biomarker [96]
PCBP4 OTDLL4NB Strong Altered Expression [73]
PCM1 OTFM133C Strong Biomarker [354]
PCP4 OTM1XXYX Strong Biomarker [355]
PDAP1 OTJSWMOD Strong Biomarker [162]
PDIA6 OT8YBR17 Strong Biomarker [102]
PENK OT8P3HMP Strong Biomarker [83]
PEX7 OTM7VBRC Strong Genetic Variation [130]
PHLPP1 OTIFXW8D Strong Altered Expression [356]
PIAS1 OTZVAHZI Strong Biomarker [357]
PITX3 OTE2KT8P Strong Biomarker [358]
PKIB OT7C8GFN Strong Biomarker [262]
PLAG1 OTT9AJQY Strong Biomarker [220]
PPP1R11 OTSHYPPW Strong Biomarker [359]
PPP1R12A OT4AVU95 Strong Altered Expression [128]
PPP1R1B OTSIJMQ9 Strong Posttranslational Modification [251]
PROS1 OTXQWNOI Strong Biomarker [360]
PSG5 OTHTU98X Strong Biomarker [361]
PSME3 OTSTC4YY Strong Biomarker [362]
PTCRA OTQTO5QZ Strong Biomarker [92]
PTMA OT2W4T1M Strong Biomarker [363]
PTPN5 OT2H1KDK Strong Biomarker [364]
PTX3 OTPXHRKU Strong Biomarker [358]
PVALB OTZW1WVQ Strong Biomarker [365]
QPRT OT6SKKLL Strong Biomarker [109]
RAB11A OTC4FW0J Strong Biomarker [366]
RAN OT2TER5M Strong Biomarker [367]
RANBP2 OTFG5CVF Strong Biomarker [197]
RASD2 OTETA815 Strong Biomarker [368]
RCAN1 OT1MVXC7 Strong Biomarker [369]
RCOR1 OTREADPC Strong Biomarker [164]
REST OTLL92LQ Strong Biomarker [370]
RLN2 OTY3OG71 Strong Biomarker [371]
RNASE4 OTA5SZLC Strong Biomarker [372]
RRAS OTBBF28C Strong Altered Expression [373]
RRS1 OTTNCZN6 Strong Altered Expression [374]
RTL10 OTHGB81W Strong Altered Expression [375]
S100A14 OTVFJJ91 Strong Biomarker [344]
SBNO1 OTNX3RL0 Strong Biomarker [376]
SBNO2 OT1C6J3K Strong Biomarker [376]
SCAMP5 OT9MXA2J Strong Biomarker [377]
SERPINB6 OT7G55IK Strong Biomarker [301]
SETDB1 OTWVUA1B Strong Biomarker [249]
SH3BP2 OT90JNBS Strong Genetic Variation [163]
SH3GL3 OTX6BOCN Strong Biomarker [378]
SIGLEC7 OTNDLURR Strong Biomarker [272]
SKIL OTNBXH32 Strong Biomarker [376]
SNCB OTELSEK6 Strong Biomarker [187]
SORBS1 OTWH8762 Strong Biomarker [301]
SORCS2 OTBF3DYK Strong Biomarker [105]
SORL1 OTQ8FFNS Strong Altered Expression [379]
SOSTDC1 OTAKDNSM Strong Altered Expression [380]
SPON2 OTE7JLNM Strong Altered Expression [381]
SPTBN2 OTDMJ75N Strong Biomarker [382]
SPTLC1 OTN0Z98K Strong Altered Expression [260]
SRI OT4R3EAC Strong Biomarker [383]
SRSF6 OTGLOSYE Strong Biomarker [261]
STATH OTQHBHM9 Strong Biomarker [384]
STH OTK8ULTH Strong Genetic Variation [385]
STIM2 OTYNXAW0 Strong Altered Expression [386]
SUMO2 OT1Y5IKN Strong Biomarker [387]
SUMO3 OTTUJQJ1 Strong Biomarker [387]
SUPT4H1 OTB3UCTB Strong Biomarker [388]
SV2B OTAO5WIU Strong Altered Expression [192]
SV2C OTIH108W Strong Altered Expression [192]
SYN1 OTMNPWC1 Strong Biomarker [389]
TAC1 OTM842YW Strong Biomarker [390]
TAF4 OTPIRFEF Strong Biomarker [391]
TAL1 OTX4K6QZ Strong Genetic Variation [221]
TARBP2 OT1QQ8H3 Strong Biomarker [392]
TIMM23 OTAAS85T Strong Altered Expression [393]
TM7SF2 OTILU5S7 Strong Biomarker [22]
TMED9 OTYGAQS0 Strong Altered Expression [394]
TMEM106B OTUWA6NW Strong Biomarker [102]
TOMM40 OTZDQ29F Strong Biomarker [202]
TPPP OTCFMSUF Strong Altered Expression [394]
TSHZ1 OTYQ9ECW Strong Biomarker [395]
DUSP2 OTH54FMR Definitive Biomarker [23]
HTT OTKN8P85 Definitive Autosomal dominant [206]
MLF1 OTC5BKHU Definitive Biomarker [396]
PSMG1 OTZ5I6UM Definitive Biomarker [23]
RAB7B OT60A0E9 Definitive Biomarker [209]
------------------------------------------------------------------------------------
⏷ Show the Full List of 280 DOT(s)

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22 Circulating miR-421 Targeting Leucocytic Angiotensin Converting Enzyme 2 Is Elevated in Patients with Chronic Kidney Disease.Nephron. 2019;141(1):61-74. doi: 10.1159/000493805. Epub 2018 Oct 16.
23 Pituitary Adenylate Cyclase-Activating Polypeptide (PACAP) Enhances Hippocampal Synaptic Plasticity and Improves Memory Performance in Huntington's Disease.Mol Neurobiol. 2018 Nov;55(11):8263-8277. doi: 10.1007/s12035-018-0972-5. Epub 2018 Mar 10.
24 A soluble truncated tau species related to cognitive dysfunction and caspase-2 is elevated in the brain of Huntington's disease patients.Acta Neuropathol Commun. 2019 Jul 30;7(1):111. doi: 10.1186/s40478-019-0764-9.
25 Identification of novel genes in Hirschsprung disease pathway using whole genome expression study.J Pediatr Surg. 2012 Feb;47(2):303-7. doi: 10.1016/j.jpedsurg.2011.11.017.
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27 Treatment with the MAO-A inhibitor clorgyline elevates monoamine neurotransmitter levels and improves affective phenotypes in a mouse model of Huntington disease.Exp Neurol. 2016 Apr;278:4-10. doi: 10.1016/j.expneurol.2016.01.019. Epub 2016 Jan 26.
28 Rare homozygosity in amyotrophic lateral sclerosis suggests the contribution of recessive variants to disease genetics.J Neurol Sci. 2019 Jul 15;402:62-68. doi: 10.1016/j.jns.2019.05.006. Epub 2019 May 8.
29 Psychological aspects of pre-symptomatic testing for Machado-Joseph disease and familial amyloid polyneuropathy type I.Clin Genet. 2006 Apr;69(4):297-305. doi: 10.1111/j.1399-0004.2006.00606.x.
30 Targeting ENT1 and adenosine tone for the treatment of Huntington's disease.Hum Mol Genet. 2017 Feb 1;26(3):467-478. doi: 10.1093/hmg/ddw402.
31 Performance of a novel high sensitivity cardiac troponin I assay in asymptomatic hemodialysis patients - evidence for sex-specific differences.Clin Chem Lab Med. 2019 Jul 26;57(8):1261-1270. doi: 10.1515/cclm-2018-1176.
32 Amelioration of Huntington's disease phenotypes by Beta-Lapachone is associated with increases in Sirt1 expression, CREB phosphorylation and PGC-1 deacetylation.PLoS One. 2018 May 9;13(5):e0195968. doi: 10.1371/journal.pone.0195968. eCollection 2018.
33 Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease.Proc Natl Acad Sci U S A. 2003 Sep 2;100(18):10483-7. doi: 10.1073/pnas.1832501100. Epub 2003 Aug 20.
34 PKR activation in neurodegenerative disease.J Neuropathol Exp Neurol. 2004 Feb;63(2):97-105. doi: 10.1093/jnen/63.2.97.
35 Trehalose rescues glial cell dysfunction in striatal cultures from HD R6/1 mice at early postnatal development.Mol Cell Neurosci. 2016 Jul;74:128-45. doi: 10.1016/j.mcn.2016.05.002. Epub 2016 May 26.
36 Relationship Between Leptin Level, Inflammation, and Volume Status in Maintenance Hemodialysis Patients.Ther Apher Dial. 2019 Feb;23(1):59-64. doi: 10.1111/1744-9987.12750. Epub 2018 Sep 18.
37 Up-regulation of the isoenzymes MAO-A and MAO-B in the human basal ganglia and pons in Huntington's disease revealed by quantitative enzyme radioautography.Brain Res. 2011 Jan 25;1370:204-14. doi: 10.1016/j.brainres.2010.11.020. Epub 2010 Nov 11.
38 Activation of NPY-Y2 receptors ameliorates disease pathology in the R6/2 mouse and PC12 cell models of Huntington's disease.Exp Neurol. 2018 Apr;302:112-128. doi: 10.1016/j.expneurol.2018.01.001. Epub 2018 Jan 6.
39 Relationship between plasma levels of sclerostin, calcium-phosphate disturbances, established markers of bone turnover, and inflammation in haemodialysis patients.Int Urol Nephrol. 2019 Mar;51(3):519-526. doi: 10.1007/s11255-018-2050-3. Epub 2018 Dec 24.
40 Association Between Toll-Like Receptor 4 (TLR4) and Triggering Receptor Expressed on Myeloid Cells 2 (TREM2) Genetic Variants and Clinical Progression of Huntington's Disease.Mov Disord. 2020 Mar;35(3):401-408. doi: 10.1002/mds.27911. Epub 2019 Nov 14.
41 TRPC5 channel instability induced by depalmitoylation protects striatal neurons against oxidative stress in Huntington's disease.Biochim Biophys Acta Mol Cell Res. 2020 Feb;1867(2):118620. doi: 10.1016/j.bbamcr.2019.118620. Epub 2019 Dec 6.
42 HAP1 is an in vivo UBE3A target that augments autophagy in a mouse model of Angelman syndrome.Neurobiol Dis. 2019 Dec;132:104585. doi: 10.1016/j.nbd.2019.104585. Epub 2019 Aug 21.
43 Genomic organization and expression analysis of mouse kynurenine aminotransferase II, a possible factor in the pathophysiology of Huntington's disease.Mamm Genome. 1999 Sep;10(9):845-52. doi: 10.1007/s003359901102.
44 Inhibitors of GABA metabolism: implications for Huntington's disease.Ann Neurol. 1977 Oct;2(4):299-303. doi: 10.1002/ana.410020407.
45 Altered Aconitase 2 Activity in Huntington's Disease Peripheral Blood Cells and Mouse Model Striatum.Int J Mol Sci. 2017 Nov 21;18(11):2480. doi: 10.3390/ijms18112480.
46 Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice.Sci Rep. 2017 Oct 27;7(1):14275. doi: 10.1038/s41598-017-14290-3.
47 Increased 5-methylcytosine and decreased 5-hydroxymethylcytosine levels are associated with reduced striatal A2AR levels in Huntington's disease.Neuromolecular Med. 2013 Jun;15(2):295-309. doi: 10.1007/s12017-013-8219-0. Epub 2013 Feb 6.
48 Genetics Modulate Gray Matter Variation Beyond Disease Burden in Prodromal Huntington's Disease.Front Neurol. 2018 Mar 29;9:190. doi: 10.3389/fneur.2018.00190. eCollection 2018.
49 Potential Correlation Between Circulating Fetuin-A and Pentraxin-3 With Biochemical Parameters of Calcification in Hemodialysis Patients.Arch Iran Med. 2017 Dec 1;20(12):752-755.
50 Prevention of cytosolic IAPs degradation: a potential pharmacological target in Huntington's Disease.Pharmacol Res. 2005 Aug;52(2):140-50. doi: 10.1016/j.phrs.2005.01.006.
51 Vascular adhesion protein-1 in hemodialysis and hemodiafiltration patients: effect of single hemodialysis session on its level in regard to type of anticoagulant.Int Urol Nephrol. 2017 May;49(5):875-879. doi: 10.1007/s11255-016-1497-3. Epub 2017 Jan 2.
52 Reducing canonical Wingless/Wnt signaling pathway confers protection against mutant Huntingtin toxicity in Drosophila.Neurobiol Dis. 2012 Aug;47(2):237-47. doi: 10.1016/j.nbd.2012.04.007. Epub 2012 Apr 17.
53 Effect of Apolipoprotein E Genotypes on Huntington's Disease Phenotypes in a Han Chinese Population.Neurosci Bull. 2019 Aug;35(4):756-762. doi: 10.1007/s12264-019-00360-1. Epub 2019 Mar 18.
54 Huntington's disease biomarker progression profile identified by transcriptome sequencing in peripheral blood.Eur J Hum Genet. 2015 Oct;23(10):1349-56. doi: 10.1038/ejhg.2014.281. Epub 2015 Jan 28.
55 Immunohistochemical analysis of huntingtin-associated protein 1 in adult rat spinal cord and its regional relationship with androgen receptor.Neuroscience. 2017 Jan 6;340:201-217. doi: 10.1016/j.neuroscience.2016.10.053. Epub 2016 Oct 29.
56 Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism.Hum Mol Genet. 2008 Oct 15;17(20):3223-35. doi: 10.1093/hmg/ddn218. Epub 2008 Jul 24.
57 The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients.PLoS One. 2013 Jul 22;8(7):e68951. doi: 10.1371/journal.pone.0068951. Print 2013.
58 Evaluating the SERCA2 and VEGF mRNAs as Potential Molecular Biomarkers of the Onset and Progression in Huntington's Disease.PLoS One. 2015 Apr 27;10(4):e0125259. doi: 10.1371/journal.pone.0125259. eCollection 2015.
59 Effects of simvastatin on neuroprotection and modulation of Bcl-2 and BAX in the rat quinolinic acid model of Huntington's disease.Neurosci Lett. 2008 Dec 19;448(1):166-9. doi: 10.1016/j.neulet.2008.10.023. Epub 2008 Oct 14.
60 Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease.Exp Neurol. 1999 Oct;159(2):362-76. doi: 10.1006/exnr.1999.7170.
61 CAG repeat disorder models and human neuropathology: similarities and differences.Acta Neuropathol. 2008 Jan;115(1):71-86. doi: 10.1007/s00401-007-0287-5. Epub 2007 Sep 5.
62 Identification of a novel caspase cleavage site in huntingtin that regulates mutant huntingtin clearance.FASEB J. 2019 Mar;33(3):3190-3197. doi: 10.1096/fj.201701510RRR. Epub 2018 Nov 13.
63 Hyperhomocysteinaemia in treated patients with Huntington's disease homocysteine in HD.Mov Disord. 2004 Feb;19(2):226-8. doi: 10.1002/mds.10629.
64 CD200 is up-regulated in R6/1 transgenic mouse model of Huntington's disease.PLoS One. 2019 Dec 2;14(12):e0224901. doi: 10.1371/journal.pone.0224901. eCollection 2019.
65 Cyclin-Dependent Kinase 5 Dysfunction Contributes to Depressive-like Behaviors in Huntington's Disease by Altering the DARPP-32 Phosphorylation Status in the Nucleus Accumbens.Biol Psychiatry. 2019 Aug 1;86(3):196-207. doi: 10.1016/j.biopsych.2019.03.001. Epub 2019 Mar 13.
66 Smaller low-density lipoprotein size as a possible risk factor for the prevalence of coronary artery diseases in haemodialysis patients: associations of cholesteryl ester transfer protein and the hepatic lipase gene polymorphism with low-density lipoprotein size.Nephrology (Carlton). 2011 Aug;16(6):558-66. doi: 10.1111/j.1440-1797.2011.01454.x.
67 Thecalretinin interneurons of the striatum: comparisons between rodents and primates under normal and pathological conditions.J Neural Transm (Vienna). 2018 Mar;125(3):279-290. doi: 10.1007/s00702-017-1687-x. Epub 2017 Feb 6.
68 Epigenetic regulation of cholinergic receptor M1 (CHRM1) by histone H3K9me3 impairs Ca(2+) signaling in Huntington's disease.Acta Neuropathol. 2013 May;125(5):727-39. doi: 10.1007/s00401-013-1103-z. Epub 2013 Mar 2.
69 Progressive Cl- channel defects reveal disrupted skeletal muscle maturation in R6/2 Huntington's mice.J Gen Physiol. 2017 Jan;149(1):55-74. doi: 10.1085/jgp.201611603. Epub 2016 Nov 29.
70 Somatostatin and cannabinoid receptors crosstalk in protection of huntingtin knock-in striatal neuronal cells in response to quinolinic acid.Neurochem Int. 2019 Oct;129:104518. doi: 10.1016/j.neuint.2019.104518. Epub 2019 Aug 2.
71 Prospects for cannabinoid therapies in basal ganglia disorders.Br J Pharmacol. 2011 Aug;163(7):1365-78. doi: 10.1111/j.1476-5381.2011.01365.x.
72 Decreased Lin7b expression in layer 5 pyramidal neurons may contribute to impaired corticostriatal connectivity in huntington disease.J Neuropathol Exp Neurol. 2010 Sep;69(9):880-95. doi: 10.1097/NEN.0b013e3181ed7a41.
73 Neuroprotective effects of psychotropic drugs in Huntington's disease.Int J Mol Sci. 2013 Nov 15;14(11):22558-603. doi: 10.3390/ijms141122558.
74 Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease.Hum Mol Genet. 2012 Aug 1;21(15):3474-88. doi: 10.1093/hmg/dds178. Epub 2012 May 15.
75 High-resolution respirometry of fine-needle muscle biopsies in pre-manifest Huntington's disease expansion mutation carriers shows normal mitochondrial respiratory function.PLoS One. 2017 Apr 13;12(4):e0175248. doi: 10.1371/journal.pone.0175248. eCollection 2017.
76 Two-track virtual screening approach to identify both competitive and allosteric inhibitors of human small C-terminal domain phosphatase 1.J Comput Aided Mol Des. 2017 Aug;31(8):743-753. doi: 10.1007/s10822-017-0037-2. Epub 2017 Jun 26.
77 CYP46A1 gene therapy deciphers the role of brain cholesterol metabolism in Huntington's disease.Brain. 2019 Aug 1;142(8):2432-2450. doi: 10.1093/brain/awz174.
78 The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin.Brain. 2018 May 1;141(5):1434-1454. doi: 10.1093/brain/awy057.
79 RTP801 Is Involved in Mutant Huntingtin-Induced Cell Death.Mol Neurobiol. 2016 Jul;53(5):2857-2868. doi: 10.1007/s12035-015-9166-6. Epub 2015 Apr 16.
80 2,4 DNP improves motor function, preserves medium spiny neuronal identity, and reduces oxidative stress in a mouse model of Huntington's disease.Exp Neurol. 2017 Jul;293:83-90. doi: 10.1016/j.expneurol.2017.03.020. Epub 2017 Mar 28.
81 Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex.EMBO J. 2018 Jan 17;37(2):282-299. doi: 10.15252/embj.201797212. Epub 2017 Dec 6.
82 Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain. Hum Mol Genet. 2014 Aug 1;23(15):4051-63. doi: 10.1093/hmg/ddu119. Epub 2014 Mar 14.
83 Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry.J Neurosci. 2008 Sep 24;28(39):9723-31. doi: 10.1523/JNEUROSCI.3044-08.2008.
84 Transcriptional correlates of the pathological phenotype in a Huntington's disease mouse model.Sci Rep. 2019 Dec 10;9(1):18696. doi: 10.1038/s41598-019-55177-9.
85 DRD3 variation associates with early-onset heroin dependence, but not specific personality traits.Prog Neuropsychopharmacol Biol Psychiatry. 2014 Jun 3;51:1-8. doi: 10.1016/j.pnpbp.2013.12.018. Epub 2014 Jan 5.
86 Localization of the D5 dopamine receptor gene to human chromosome 4p15.1-p15.3, centromeric to the Huntington's disease locus.Genomics. 1992 Mar;12(3):510-6. doi: 10.1016/0888-7543(92)90442-u.
87 MAP kinase phosphatase 1 (MKP-1/DUSP1) is neuroprotective in Huntington's disease via additive effects of JNK and p38 inhibition.J Neurosci. 2013 Feb 6;33(6):2313-25. doi: 10.1523/JNEUROSCI.4965-11.2013.
88 Cell cycle activation in striatal neurons from Huntington's disease patients and rats treated with 3-nitropropionic acid.Int J Dev Neurosci. 2008 Nov;26(7):665-71. doi: 10.1016/j.ijdevneu.2008.07.016. Epub 2008 Aug 12.
89 Exploring Genetic Factors Involved in Huntington Disease Age of Onset: E2F2 as a New Potential Modifier Gene.PLoS One. 2015 Jul 6;10(7):e0131573. doi: 10.1371/journal.pone.0131573. eCollection 2015.
90 Correction of Huntington's Disease Phenotype by Genistein-Induced Autophagy in the Cellular Model.Neuromolecular Med. 2018 Mar;20(1):112-123. doi: 10.1007/s12017-018-8482-1. Epub 2018 Feb 12.
91 Remodeling of heterochromatin structure slows neuropathological progression and prolongs survival in an animal model of Huntington's disease.Acta Neuropathol. 2017 Nov;134(5):729-748. doi: 10.1007/s00401-017-1732-8. Epub 2017 Jun 7.
92 Dysregulated brain creatine kinase is associated with hearing impairment in mouse models of Huntington disease.J Clin Invest. 2011 Apr;121(4):1519-23. doi: 10.1172/JCI43220. Epub 2011 Mar 14.
93 Cross linking of polyglutamine domains catalyzed by tissue transglutaminase is greatly favored with pathological-length repeats: does transglutaminase activity play a role in (CAG)(n)/Q(n)-expansion diseases?.Neurochem Int. 2002 Jan;40(1):53-67. doi: 10.1016/s0197-0186(01)00058-4.
94 Longitudinal assessment of the Unified Huntington's Disease Rating Scale (UHDRS) and UHDRS-For Advanced Patients (UHDRS-FAP) in patients with late stage Huntington's disease.Eur J Neurol. 2019 May;26(5):780-785. doi: 10.1111/ene.13889. Epub 2019 Jan 27.
95 Differential Impacts of Intravenous Iron Administration and Iron-Containing Phosphate Binders on Serum Intact Fibroblast Growth Factor 23 Levels.Blood Purif. 2019;47 Suppl 2:63-69. doi: 10.1159/000496640. Epub 2019 Apr 3.
96 Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)-binding protein 1 in Huntington's disease.Neuropathology. 2019 Oct;39(5):358-367. doi: 10.1111/neup.12600. Epub 2019 Oct 9.
97 A GABBR2 gene variant modifies pathophysiology in Huntington's disease.Neurosci Lett. 2016 May 4;620:8-13. doi: 10.1016/j.neulet.2016.03.038. Epub 2016 Mar 23.
98 Palmitoylation and trafficking of GAD65 are impaired in a cellular model of Huntington's disease.Biochem J. 2012 Feb 15;442(1):39-48. doi: 10.1042/BJ20110679.
99 Mutant huntingtin reduction in astrocytes slows disease progression in the BACHD conditional Huntington's disease mouse model.Hum Mol Genet. 2019 Feb 1;28(3):487-500. doi: 10.1093/hmg/ddy363.
100 Bilateral quinolinic acid-induced lipid peroxidation, decreased striatal monoamine levels and neurobehavioral deficits are ameliorated by GIP receptor agonist D-Ala(2)GIP in rat model of Huntington's disease.Eur J Pharmacol. 2018 Jun 5;828:31-41. doi: 10.1016/j.ejphar.2018.03.034. Epub 2018 Mar 22.
101 Genetic predisposition to advanced glycation end products toxicity is related to prognosis of chronic hemodialysis patients.Kidney Blood Press Res. 2010;33(1):30-6. doi: 10.1159/000285845. Epub 2010 Feb 23.
102 Epigenetic dysregulation of hairy and enhancer of split 4 (HES4) is associated with striatal degeneration in postmortem Huntington brains.Hum Mol Genet. 2015 Mar 1;24(5):1441-56. doi: 10.1093/hmg/ddu561. Epub 2014 Dec 5.
103 Targeting Gpr52 lowers mutant HTT levels and rescues Huntington's disease-associated phenotypes.Brain. 2018 Jun 1;141(6):1782-1798. doi: 10.1093/brain/awy081.
104 Mapping of the human NMDA receptor subunit (NMDAR1) and the proposed NMDA receptor glutamate-binding subunit (NMDARA1) to chromosomes 9q34.3 and chromosome 8, respectively.Genomics. 1993 Jul;17(1):237-9. doi: 10.1006/geno.1993.1311.
105 SorCS2-mediated NR2A trafficking regulates motor deficits in Huntington's disease.JCI Insight. 2017 May 4;2(9):e88995. doi: 10.1172/jci.insight.88995. eCollection 2017 May 4.
106 Rapid homeostatic downregulation of LTP by extrasynaptic GluN2B receptors.J Neurophysiol. 2018 Nov 1;120(5):2351-2357. doi: 10.1152/jn.00421.2018. Epub 2018 Aug 15.
107 Autophagy is increased following either pharmacological or genetic silencing of mGluR5 signaling in Alzheimer's disease mouse models.Mol Brain. 2018 Apr 10;11(1):19. doi: 10.1186/s13041-018-0364-9.
108 Nerve growth factor and striatal glutathione metabolism in a rat model of Huntington's disease.Restor Neurol Neurosci. 2000;17(4):217-221.
109 Basal ganglia lesions in the rat: effects on quinolinic acid metabolism.Brain Res. 1989 Jun 19;490(1):103-9. doi: 10.1016/0006-8993(89)90435-6.
110 Association of Hepcidin With Anemia Parameters in Incident Dialysis Patients: Differences Between Dialysis Modalities.Ther Apher Dial. 2020 Feb;24(1):4-16. doi: 10.1111/1744-9987.12837. Epub 2019 Jun 10.
111 Hypothalamic alterations in Huntington's disease patients: comparison with genetic rodent models.J Neuroendocrinol. 2014 Nov;26(11):761-75. doi: 10.1111/jne.12190.
112 Histone deacetylase-1 (HDAC1) is a molecular switch between neuronal survival and death.J Biol Chem. 2012 Oct 12;287(42):35444-35453. doi: 10.1074/jbc.M112.394544. Epub 2012 Aug 23.
113 A selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington's disease mice.Sci Rep. 2017 Jul 20;7(1):6082. doi: 10.1038/s41598-017-05125-2.
114 Hdac4 Interactions in Huntington's Disease Viewed Through the Prism of Multiomics.Mol Cell Proteomics. 2019 Aug 9;18(8 suppl 1):S92-S113. doi: 10.1074/mcp.RA118.001253. Epub 2019 Apr 30.
115 Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease.Hum Mol Genet. 2008 Dec 1;17(23):3767-75. doi: 10.1093/hmg/ddn273. Epub 2008 Sep 1.
116 Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1 Transcription Factors.Front Cell Neurosci. 2019 Mar 19;13:103. doi: 10.3389/fncel.2019.00103. eCollection 2019.
117 Perospirone in treatment of Huntington's disease: a first case report.Prog Neuropsychopharmacol Biol Psychiatry. 2007 Jan 30;31(1):308-10. doi: 10.1016/j.pnpbp.2006.06.021. Epub 2006 Aug 1.
118 Misfolded protein aggregates: mechanisms, structures and potential for disease transmission.Semin Cell Dev Biol. 2011 Jul;22(5):482-7. doi: 10.1016/j.semcdb.2011.04.002. Epub 2011 May 5.
119 Linkage, but not gene order, of homologous loci, including alpha-L-iduronidase (Idua), is conserved in the Huntington disease region of the mouse and human genomes.Mamm Genome. 1992;3(1):23-7. doi: 10.1007/BF00355837.
120 Interferon-3 as a Predictor of Survival in Hemodialysis Patients.Curr Mol Med. 2018;18(4):207-215. doi: 10.2174/1566524018666180926162324.
121 Polymorphisms of T helper cell cytokine-associated genes and survival of hemodialysis patients - a prospective study.BMC Nephrol. 2017 May 19;18(1):165. doi: 10.1186/s12882-017-0582-x.
122 Relationships between interleukin-12B and interleukin-10 gene polymorphisms and hepatitis C in Chinese Han hemodialysis patients.Ren Fail. 2015 Apr;37(3):505-10. doi: 10.3109/0886022X.2015.1006086. Epub 2015 Jan 23.
123 Aberrant calcium signaling by transglutaminase-mediated posttranslational modification of inositol 1,4,5-trisphosphate receptors.Proc Natl Acad Sci U S A. 2014 Sep 23;111(38):E3966-75. doi: 10.1073/pnas.1409730111. Epub 2014 Sep 8.
124 Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxias.Neurochem Res. 2011 Jul;36(7):1186-97. doi: 10.1007/s11064-010-0393-y. Epub 2011 Jan 6.
125 Targeting H3K4 trimethylation in Huntington disease.Proc Natl Acad Sci U S A. 2013 Aug 6;110(32):E3027-36. doi: 10.1073/pnas.1311323110. Epub 2013 Jul 19.
126 Development of a Rapid Fluorescence-Based High-Throughput Screening Assay to Identify Novel Kynurenine 3-Monooxygenase Inhibitor Scaffolds.SLAS Discov. 2018 Jul;23(6):554-560. doi: 10.1177/2472555218757180. Epub 2018 Feb 8.
127 Transspinal delivery of drugs by transdermal patch back-of-neck for Alzheimer's disease: a new route of administration.Discov Med. 2019 Jan;27(146):37-43.
128 Rho Kinase Pathway Alterations in the Brain and Leukocytes in Huntington's Disease.Mol Neurobiol. 2016 May;53(4):2132-40. doi: 10.1007/s12035-015-9147-9. Epub 2015 May 5.
129 Vitamin E suppresses 5-lipoxygenase-mediated oxidative stress in peripheral blood mononuclear cells of hemodialysis patients regardless of administration route. Am J Kidney Dis. 2001 May;37(5):964-9. doi: 10.1016/s0272-6386(05)80012-5.
130 ASK1 and MAP2K6 as modifiers of age at onset in Huntington's disease.J Mol Med (Berl). 2008 Apr;86(4):485-90. doi: 10.1007/s00109-007-0299-6. Epub 2008 Mar 8.
131 Implication of the JNK pathway in a rat model of Huntington's disease.Exp Neurol. 2009 Jan;215(1):191-200. doi: 10.1016/j.expneurol.2008.10.008. Epub 2008 Oct 28.
132 Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin.Nat Neurosci. 2009 Jul;12(7):864-71. doi: 10.1038/nn.2346. Epub 2009 Jun 14.
133 Suppression of MAPK11 or HIPK3 reduces mutant Huntingtin levels in Huntington's disease models.Cell Res. 2017 Dec;27(12):1441-1465. doi: 10.1038/cr.2017.113. Epub 2017 Oct 13.
134 Microstructural changes observed with DKI in a transgenic Huntington rat model: evidence for abnormal neurodevelopment.Neuroimage. 2012 Jan 16;59(2):957-67. doi: 10.1016/j.neuroimage.2011.08.062. Epub 2011 Aug 30.
135 The p. R151C Polymorphism in MC1R Gene Modifies the Age of Onset in Spanish Huntington's Disease Patients.Mol Neurobiol. 2017 Jul;54(5):3906-3910. doi: 10.1007/s12035-016-0305-5. Epub 2016 Dec 6.
136 Significance of serum Myostatin in hemodialysis patients.BMC Nephrol. 2019 Dec 11;20(1):462. doi: 10.1186/s12882-019-1647-9.
137 Astrocyte elevated gene-1 induces autophagy in diabetic cardiomyopathy through upregulation of KLF4.J Cell Biochem. 2019 Jun;120(6):9709-9715. doi: 10.1002/jcb.28249. Epub 2018 Dec 5.
138 The melatonin MT1 receptor axis modulates mutant Huntingtin-mediated toxicity.J Neurosci. 2011 Oct 12;31(41):14496-507. doi: 10.1523/JNEUROSCI.3059-11.2011.
139 Risk Factors for Primary Arteriovenous Fistula Dysfunction in Hemodialysis Patients: A Retrospective Survival Analysis in Multiple Medical Centers.Blood Purif. 2019;48(3):276-282. doi: 10.1159/000500045. Epub 2019 May 8.
140 Gintonin, a ginseng-derived ingredient, as a novel therapeutic strategy for Huntington's disease: Activation of the Nrf2 pathway through lysophosphatidic acid receptors.Brain Behav Immun. 2019 Aug;80:146-162. doi: 10.1016/j.bbi.2019.03.001. Epub 2019 Mar 7.
141 TSPO-PET imaging using [18F]PBR06 is a potential translatable biomarker for treatment response in Huntington's disease: preclinical evidence with the p75NTR ligand LM11A-31.Hum Mol Genet. 2018 Aug 15;27(16):2893-2912. doi: 10.1093/hmg/ddy202.
142 Development of 2-thioxoquinazoline-4-one derivatives as dual and selective inhibitors of dynamin-related protein 1 (Drp1) and puromycin-sensitive aminopeptidase (PSA).Chem Pharm Bull (Tokyo). 2014;62(10):979-88. doi: 10.1248/cpb.c14-00333.
143 Association of age at onset in Huntington disease with functional promoter variations in NPY and NPY2R.J Mol Med (Berl). 2014 Feb;92(2):177-84. doi: 10.1007/s00109-013-1092-3.
144 Calcium-sensing receptor gene (CASR) polymorphisms and CASR transcript level concerning dyslipidemia in hemodialysis patients: a cross-sectional study.BMC Nephrol. 2019 Nov 27;20(1):436. doi: 10.1186/s12882-019-1619-0.
145 Do BDNF and NT-4/5 exert synergistic or occlusive effects on corticostriatal transmission in a male mouse model of Huntington's disease?.J Neurosci Res. 2019 Dec;97(12):1665-1677. doi: 10.1002/jnr.24507. Epub 2019 Aug 7.
146 High and Low Levels of an NTRK2-Driven Genetic Profile Affect Motor- and Cognition-Associated Frontal Gray Matter in Prodromal Huntington's Disease.Brain Sci. 2018 Jun 22;8(7):116. doi: 10.3390/brainsci8070116.
147 The impact of single-nucleotide polymorphisms (SNPs) in OGG1 and XPC on the age at onset of Huntington disease.Mutat Res. 2013 Aug 15;755(2):115-9. doi: 10.1016/j.mrgentox.2013.04.020. Epub 2013 Jul 2.
148 CRISPR/Cas9 Editing of the Mutant Huntingtin Allele InVitro and InVivo.Mol Ther. 2017 Jan 4;25(1):12-23. doi: 10.1016/j.ymthe.2016.11.010. Epub 2017 Jan 4.
149 PDE10A mutations help to unwrap the neurobiology of hyperkinetic disorders.Cell Signal. 2019 Aug;60:31-38. doi: 10.1016/j.cellsig.2019.04.001. Epub 2019 Apr 2.
150 Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms.Neuroscience. 2004;123(4):967-81. doi: 10.1016/j.neuroscience.2003.11.009.
151 Cyclic nucleotide signaling changes associated with normal aging and age-related diseases of the brain.Cell Signal. 2018 Jan;42:281-291. doi: 10.1016/j.cellsig.2017.11.004. Epub 2017 Nov 23.
152 Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease.J Neurosci. 2017 Mar 8;37(10):2776-2794. doi: 10.1523/JNEUROSCI.2006-14.2016. Epub 2017 Jan 25.
153 The complexity of reproductive decision-making in asymptomatic carriers of the Huntington mutation.Eur J Hum Genet. 2007 Apr;15(4):453-62. doi: 10.1038/sj.ejhg.5201774. Epub 2007 Jan 24.
154 Effects of peptidyl-prolyl isomerase 1 depletion in animal models of prion diseases.Prion. 2018 Mar 4;12(2):127-137. doi: 10.1080/19336896.2018.1464367. Epub 2018 May 18.
155 Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription.Elife. 2019 Apr 17;8:e42988. doi: 10.7554/eLife.42988.
156 Characterization of adenine nucleotide metabolism in the cellular model of Huntington's disease.Nucleosides Nucleotides Nucleic Acids. 2018;37(11):630-638. doi: 10.1080/15257770.2018.1481508. Epub 2018 Dec 27.
157 Erucic acid, a nutritional PPAR-ligand may influence Huntington's disease pathogenesis.Metab Brain Dis. 2020 Jan;35(1):1-9. doi: 10.1007/s11011-019-00500-6. Epub 2019 Oct 17.
158 Amelioration of Huntington's disease phenotype in astrocytes derived from iPSC-derived neural progenitor cells of Huntington's disease monkeys.PLoS One. 2019 Mar 21;14(3):e0214156. doi: 10.1371/journal.pone.0214156. eCollection 2019.
159 Putting proteins in their place: palmitoylation in Huntington disease and other neuropsychiatric diseases.Prog Neurobiol. 2012 May;97(2):220-38. doi: 10.1016/j.pneurobio.2011.11.002. Epub 2011 Dec 7.
160 Impact of Weekly Teriparatide on the Bone and Mineral Metabolism in Hemodialysis Patients With Relatively Low Serum Parathyroid Hormone: A Pilot Study.Ther Apher Dial. 2020 Apr;24(2):146-153. doi: 10.1111/1744-9987.12867. Epub 2019 Jul 21.
161 siRNA screen identifies QPCT as a druggable target for Huntington's disease.Nat Chem Biol. 2015 May;11(5):347-354. doi: 10.1038/nchembio.1790. Epub 2015 Apr 6.
162 Infrainguinal bypass surgery outcomes are worse in hemodialysis patients compared with patients with renal transplants.J Vasc Surg. 2019 Mar;69(3):850-856. doi: 10.1016/j.jvs.2018.05.252. Epub 2018 Dec 21.
163 Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region.Am J Hum Genet. 2012 Mar 9;90(3):434-44. doi: 10.1016/j.ajhg.2012.01.005. Epub 2012 Mar 1.
164 MicroRNA-22 (miR-22) overexpression is neuroprotective via general anti-apoptotic effects and may also target specific Huntington's disease-related mechanisms.PLoS One. 2013;8(1):e54222. doi: 10.1371/journal.pone.0054222. Epub 2013 Jan 17.
165 Dysregulation of receptor interacting protein-2 and caspase recruitment domain only protein mediates aberrant caspase-1 activation in Huntington's disease.J Neurosci. 2005 Dec 14;25(50):11645-54. doi: 10.1523/JNEUROSCI.4181-05.2005.
166 Loss of SNAP-25 and rabphilin 3a in sensory-motor cortex in Huntington's disease.J Neurochem. 2007 Oct;103(1):115-23. doi: 10.1111/j.1471-4159.2007.04703.x.
167 Loss of striatal 90-kDa ribosomal S6 kinase (Rsk) is a key factor for motor, synaptic and transcription dysfunction in Huntington's disease.Biochim Biophys Acta. 2016 Jul;1862(7):1255-66. doi: 10.1016/j.bbadis.2016.04.002. Epub 2016 Apr 6.
168 Associations between Soluble Receptor for Advanced Glycation End Products (sRAGE) and S100A12 (EN-RAGE) with Mortality in Long-term Hemodialysis Patients.J Korean Med Sci. 2017 Jan;32(1):54-59. doi: 10.3346/jkms.2017.32.1.54.
169 Stimulation of S1PR5 with A-971432, a selective agonist, preserves blood-brain barrier integrity and exerts therapeutic effect in an animal model of Huntington's disease.Hum Mol Genet. 2018 Jul 15;27(14):2490-2501. doi: 10.1093/hmg/ddy153.
170 A genetic modifier suggests that endurance exercise exacerbates Huntington's disease.Hum Mol Genet. 2018 May 15;27(10):1723-1731. doi: 10.1093/hmg/ddy077.
171 Traditional Chinese medicine on four patients with Huntington's disease.Mov Disord. 2009 Feb 15;24(3):453-5. doi: 10.1002/mds.22447.
172 Identifying therapeutic targets by combining transcriptional data with ordinal clinical measurements.Nat Commun. 2017 Sep 20;8(1):623. doi: 10.1038/s41467-017-00353-6.
173 Pridopidine stabilizes mushroom spines in mouse models of Alzheimer's disease by acting on the sigma-1 receptor.Neurobiol Dis. 2019 Apr;124:489-504. doi: 10.1016/j.nbd.2018.12.022. Epub 2018 Dec 27.
174 SIRT1 is increased in affected brain regions and hypothalamic metabolic pathways are altered in Huntington disease.Neuropathol Appl Neurobiol. 2019 Jun;45(4):361-379. doi: 10.1111/nan.12514. Epub 2018 Aug 29.
175 Mitochondrial SIRT3 and neurodegenerative brain disorders.J Chem Neuroanat. 2019 Jan;95:43-53. doi: 10.1016/j.jchemneu.2017.11.009. Epub 2017 Nov 9.
176 Expression of RNAs Coding for Metal Transporters in Blood of Patients with Huntington's Disease.Neurochem Res. 2016 Feb;41(1-2):101-6. doi: 10.1007/s11064-015-1737-4. Epub 2015 Oct 15.
177 Enhanced Na(+) -K(+) -2Cl(-) cotransporter 1 underlies motor dysfunction in huntington's disease.Mov Disord. 2019 Jun;34(6):845-857. doi: 10.1002/mds.27651. Epub 2019 Mar 6.
178 Restoring GABAergic inhibition rescues memory deficits in a Huntington's disease mouse model.Proc Natl Acad Sci U S A. 2018 Feb 13;115(7):E1618-E1626. doi: 10.1073/pnas.1716871115. Epub 2018 Jan 30.
179 Deutetrabenazine for the treatment of Huntington's chorea.Expert Rev Neurother. 2018 Aug;18(8):625-631. doi: 10.1080/14737175.2018.1500178. Epub 2018 Jul 17.
180 Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease.J Neurosci. 2010 Mar 31;30(13):4552-61. doi: 10.1523/JNEUROSCI.5865-09.2010.
181 Post-translational Regulation of GLT-1 in Neurological Diseases and Its Potential as an Effective Therapeutic Target.Front Mol Neurosci. 2019 Jul 9;12:164. doi: 10.3389/fnmol.2019.00164. eCollection 2019.
182 Striatal excitatory amino acid transporter transcript expression in schizophrenia, bipolar disorder, and major depressive disorder.Neuropsychopharmacology. 2002 Mar;26(3):368-75. doi: 10.1016/S0893-133X(01)00370-0.
183 Defective regulation of iron transporters leading to iron excess in the polymorphonuclear leukocytes of patients on maintenance hemodialysis.Am J Kidney Dis. 2004 Jun;43(6):1030-9. doi: 10.1053/j.ajkd.2004.02.016.
184 Mesenchymal stem cells alleviate AQP-4-dependent glymphatic dysfunction and improve brain distribution of antisense oligonucleotides in BACHD mice.Stem Cells. 2020 Feb;38(2):218-230. doi: 10.1002/stem.3103. Epub 2019 Oct 31.
185 Influence of SLCO1B3 gene polymorphism on the pharmacokinetics of digoxin in terminal renal failure.Drug Metab Pharmacokinet. 2008;23(6):406-11. doi: 10.2133/dmpk.23.406.
186 Co-occurrence of mixed proteinopathies in late-stage Huntington's disease.Acta Neuropathol. 2018 Feb;135(2):249-265. doi: 10.1007/s00401-017-1786-7. Epub 2017 Nov 13.
187 The therapeutic potential of intrabodies in neurologic disorders: focus on Huntington and Parkinson diseases.BioDrugs. 2006;20(6):327-33. doi: 10.2165/00063030-200620060-00002.
188 Mutant Huntingtin Affects Diabetes and Alzheimer's Markers in Human and Cell Models of Huntington's Disease.Cells. 2019 Aug 23;8(9):962. doi: 10.3390/cells8090962.
189 Inhibiting sphingosine kinase 2 mitigates mutant Huntingtin-induced neurodegeneration in neuron models of Huntington disease.Hum Mol Genet. 2017 Apr 1;26(7):1305-1317. doi: 10.1093/hmg/ddx046.
190 Striatal GABAergic interneuron dysfunction in the Q175 mouse model of Huntington's disease.Eur J Neurosci. 2019 Jan;49(1):79-93. doi: 10.1111/ejn.14283. Epub 2018 Dec 10.
191 Studies of a DNA marker (G8) genetically linked to Huntington disease in British families.Hum Genet. 1986 Aug;73(4):333-9. doi: 10.1007/BF00279096.
192 Mutant Huntingtin Causes a Selective Decrease in the Expression of Synaptic Vesicle Protein 2C.Neurosci Bull. 2018 Oct;34(5):747-758. doi: 10.1007/s12264-018-0230-x. Epub 2018 Apr 30.
193 Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase.J Clin Invest. 2006 May;116(5):1410-24. doi: 10.1172/JCI27607. Epub 2006 Apr 6.
194 Validating Body Fat Assessment by Bioelectric Impedance Spectroscopy in Taiwanese Hemodialysis Patients.J Ren Nutr. 2017 Jan;27(1):37-44. doi: 10.1053/j.jrn.2016.08.003. Epub 2016 Sep 22.
195 Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease.Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2712-7. doi: 10.1073/pnas.0437967100. Epub 2003 Feb 25.
196 Increased TRPC5 glutathionylation contributes to striatal neuron loss in Huntington's disease.Brain. 2015 Oct;138(Pt 10):3030-47. doi: 10.1093/brain/awv188. Epub 2015 Jun 30.
197 Dietary Tryptophan Induces Opposite Health-Related Responses in the Senegalese Sole (Solea senegalensis) Reared at Low or High Stocking Densities With Implications in Disease Resistance.Front Physiol. 2019 May 1;10:508. doi: 10.3389/fphys.2019.00508. eCollection 2019.
198 A two years longitudinal study of a transgenic Huntington disease monkey.BMC Neurosci. 2014 Mar 3;15:36. doi: 10.1186/1471-2202-15-36.
199 Polymorphism of HD and UCHL-1 genes in Huntington's disease.J Clin Neurosci. 2009 Nov;16(11):1473-7. doi: 10.1016/j.jocn.2009.03.027. Epub 2009 Aug 14.
200 Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation.Hum Mol Genet. 2010 Aug 15;19(16):3190-205. doi: 10.1093/hmg/ddq229. Epub 2010 Jun 7.
201 ATAD3A oligomerization causes neurodegeneration by coupling mitochondrial fragmentation and bioenergetics defects.Nat Commun. 2019 Mar 26;10(1):1371. doi: 10.1038/s41467-019-09291-x.
202 VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease.Biochim Biophys Acta Mol Basis Dis. 2017 Feb;1863(2):552-559. doi: 10.1016/j.bbadis.2016.11.026. Epub 2016 Nov 29.
203 Decreased VIP and VPAC2 receptor expression in the biological clock of the R6/2 Huntington's disease mouse.J Mol Neurosci. 2007;31(2):139-48. doi: 10.1385/jmn/31:02:139.
204 Epigenetic and transcriptional modulation of WDR5, a chromatin remodeling protein, in Huntington's disease human induced pluripotent stem cell (hiPSC) model.Mol Cell Neurosci. 2017 Jul;82:46-57. doi: 10.1016/j.mcn.2017.04.013. Epub 2017 May 2.
205 Atypical ubiquitination by E3 ligase WWP1 inhibits the proteasome-mediated degradation of mutant huntingtin.Brain Res. 2016 Jul 15;1643:103-12. doi: 10.1016/j.brainres.2016.03.027. Epub 2016 Apr 21.
206 Technical standards for the interpretation and reporting of constitutional copy-number variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics (ACMG) and the Clinical Genome Resource (ClinGen). Genet Med. 2020 Feb;22(2):245-257. doi: 10.1038/s41436-019-0686-8. Epub 2019 Nov 6.
207 Familial dyskinesia and facial myokymia (FDFM): a novel movement disorder.Ann Neurol. 2001 Apr;49(4):486-92.
208 Investigating the Contribution of NPSR1, IL-6 and BDNF Polymorphisms to Depressive and Anxiety Symptoms in Hemodialysis Patients.Prog Neuropsychopharmacol Biol Psychiatry. 2019 Aug 30;94:109657. doi: 10.1016/j.pnpbp.2019.109657. Epub 2019 May 25.
209 Rab7 may be a novel therapeutic target for neurologic diseases as a key regulator in autophagy.J Neurosci Res. 2017 Oct;95(10):1993-2004. doi: 10.1002/jnr.24034. Epub 2017 Feb 10.
210 Aerobic glycolysis in amyotrophic lateral sclerosis and Huntington's disease.Rev Neurosci. 2018 Jul 26;29(5):547-555. doi: 10.1515/revneuro-2017-0075.
211 Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically.Free Radic Biol Med. 2018 May 20;120:317-329. doi: 10.1016/j.freeradbiomed.2018.04.002. Epub 2018 Apr 4.
212 Altered lactate metabolism in Huntington's disease is dependent on GLUT3 expression.CNS Neurosci Ther. 2018 Apr;24(4):343-352. doi: 10.1111/cns.12837.
213 Na+/H+ exchangers induce autophagy in neurons and inhibit polyglutamine-induced aggregate formation.PLoS One. 2013 Nov 21;8(11):e81313. doi: 10.1371/journal.pone.0081313. eCollection 2013.
214 Characterization of Endocannabinoid-Metabolizing Enzymes in Human Peripheral Blood Mononuclear Cells under Inflammatory Conditions.Molecules. 2018 Dec 1;23(12):3167. doi: 10.3390/molecules23123167.
215 The cargo receptor SQSTM1 ameliorates neurofibrillary tangle pathology and spreading through selective targeting of pathological MAPT (microtubule associated protein tau).Autophagy. 2019 Apr;15(4):583-598. doi: 10.1080/15548627.2018.1532258. Epub 2018 Oct 16.
216 Synthetic lethal screening in the mammalian central nervous system identifies Gpx6 as a modulator of Huntington's disease.Proc Natl Acad Sci U S A. 2015 Jan 6;112(1):268-72. doi: 10.1073/pnas.1417231112. Epub 2014 Dec 22.
217 Glutathione S-Transferase Omega 1 variation does not influence age at onset of Huntington's disease.BMC Med Genet. 2004 Mar 24;5:7. doi: 10.1186/1471-2350-5-7.
218 Huntington's disease: Neural dysfunction linked to inositol polyphosphate multikinase.Proc Natl Acad Sci U S A. 2015 Aug 4;112(31):9751-6. doi: 10.1073/pnas.1511810112. Epub 2015 Jul 20.
219 Dissection and Preparation of Human Primary Fetal Ganglionic Eminence Tissue for Research and Clinical Applications.Methods Mol Biol. 2018;1780:573-583. doi: 10.1007/978-1-4939-7825-0_26.
220 Olfactory abnormalities in Huntington's disease: decreased plasticity in the primary olfactory cortex of R6/1 transgenic mice and reduced olfactory discrimination in patients.Brain Res. 2007 Jun 2;1151:219-26. doi: 10.1016/j.brainres.2007.03.018. Epub 2007 Mar 12.
221 Importance of psychiatric examination in predictive genetic testing for Huntington disease.Neurol Neurochir Pol. 2013 Nov-Dec;47(6):534-41. doi: 10.5114/ninp.2013.39070.
222 Mutant huntingtin protein: a substrate for transglutaminase 1, 2, and 3.J Neuropathol Exp Neurol. 2005 Jan;64(1):58-65. doi: 10.1093/jnen/64.1.58.
223 Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis.Cell Cycle. 2012 May 15;11(10):2006-21. doi: 10.4161/cc.20423. Epub 2012 May 15.
224 Cullin-4B E3 ubiquitin ligase mediates Apaf-1 ubiquitination to regulate caspase-9 activity.PLoS One. 2019 Jul 22;14(7):e0219782. doi: 10.1371/journal.pone.0219782. eCollection 2019.
225 Pathogenesis of SCA3 and implications for other polyglutamine diseases.Neurobiol Dis. 2020 Feb;134:104635. doi: 10.1016/j.nbd.2019.104635. Epub 2019 Oct 24.
226 Forecast post-dialysis blood pressure in hemodialysis patients with intradialytic hypertension.Clin Exp Hypertens. 2019;41(6):571-576. doi: 10.1080/10641963.2018.1523916. Epub 2018 Oct 16.
227 Dysregulation of core components of SCF complex in poly-glutamine disorders.Cell Death Dis. 2012 Nov 22;3(11):e428. doi: 10.1038/cddis.2012.166.
228 Serum Erythroferrone Levels Associate with Mortality and Cardiovascular Events in Hemodialysis and in CKD Patients: A Two Cohorts Study.J Clin Med. 2019 Apr 16;8(4):523. doi: 10.3390/jcm8040523.
229 Fibroblast growth factor 9 activates anti-oxidative functions of Nrf2 through ERK signalling in striatal cell models of Huntington's disease.Free Radic Biol Med. 2019 Jan;130:256-266. doi: 10.1016/j.freeradbiomed.2018.10.455. Epub 2018 Nov 2.
230 Structure of FBP11 WW1-PL ligand complex reveals the mechanism of proline-rich ligand recognition by group II/III WW domains.J Biol Chem. 2006 Dec 29;281(52):40321-9. doi: 10.1074/jbc.M609321200. Epub 2006 Oct 24.
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233 Transcriptional Elongation Regulator 1 Affects Transcription and Splicing of Genes Associated with Cellular Morphology and Cytoskeleton Dynamics and Is Required for Neurite Outgrowth in Neuroblastoma Cells and Primary Neuronal Cultures.Mol Neurobiol. 2017 Dec;54(10):7808-7823. doi: 10.1007/s12035-016-0284-6. Epub 2016 Nov 14.
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235 Inositol hexakisphosphate kinases induce cell death in Huntington disease.J Biol Chem. 2011 Jul 29;286(30):26680-6. doi: 10.1074/jbc.M111.220749. Epub 2011 Jun 7.
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239 Mutant huntingtin induces iron overload via up-regulating IRP1 in Huntington's disease.Cell Biosci. 2018 Jul 4;8:41. doi: 10.1186/s13578-018-0239-x. eCollection 2018.
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253 Mutant huntingtin-impaired degradation of beta-catenin causes neurotoxicity in Huntington's disease.EMBO J. 2010 Jul 21;29(14):2433-45. doi: 10.1038/emboj.2010.117. Epub 2010 Jun 8.
254 The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease.Neurobiol Dis. 2017 Jan;97(Pt A):46-59. doi: 10.1016/j.nbd.2016.10.006. Epub 2016 Nov 3.
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260 De novo Synthesis of Sphingolipids Is Defective in Experimental Models of Huntington's Disease.Front Neurosci. 2017 Dec 19;11:698. doi: 10.3389/fnins.2017.00698. eCollection 2017.
261 MAP2 Splicing is Altered in Huntington's Disease.Brain Pathol. 2017 Mar;27(2):181-189. doi: 10.1111/bpa.12387. Epub 2016 Jul 7.
262 Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells.Hum Mol Genet. 2012 Sep 1;21(17):3883-95. doi: 10.1093/hmg/dds216. Epub 2012 Jun 7.
263 Cerebrospinal fluid sCD27 levels indicate active T cell-mediated inflammation in premanifest Huntington's disease.PLoS One. 2018 Feb 23;13(2):e0193492. doi: 10.1371/journal.pone.0193492. eCollection 2018.
264 Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.Hum Mol Genet. 2007 Aug 1;16(15):1845-61. doi: 10.1093/hmg/ddm133. Epub 2007 May 21.
265 Enhancement of brain-type creatine kinase activity ameliorates neuronal deficits in Huntington's disease.Biochim Biophys Acta. 2013 Jun;1832(6):742-53. doi: 10.1016/j.bbadis.2013.02.006. Epub 2013 Feb 15.
266 Factors influencing survival time of hemodialysis patients; time to event analysis using parametric models: a cohort study.BMC Nephrol. 2019 Jun 11;20(1):215. doi: 10.1186/s12882-019-1382-2.
267 PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD).Cell Cycle. 2015;14(11):1716-29. doi: 10.1080/15384101.2015.1033595.
268 Hemodialysis patient characteristics associated with better experience as measured by the In-center Hemodialysis Consumer Assessment of Healthcare Providers and Systems (ICH CAHPS) survey.BMC Nephrol. 2018 Nov 28;19(1):340. doi: 10.1186/s12882-018-1147-3.
269 Clorgyline-mediated reversal of neurological deficits in a Complexin 2 knockout mouse.Hum Mol Genet. 2010 Sep 1;19(17):3402-12. doi: 10.1093/hmg/ddq252. Epub 2010 Jun 28.
270 Oxidative stress and plasma aminopeptidase activity in Huntington's disease.J Neural Transm (Vienna). 2010 Mar;117(3):325-32. doi: 10.1007/s00702-009-0364-0. Epub 2010 Jan 22.
271 Cockayne syndrome B protein antagonizes OGG1 in modulating CAG repeat length in vivo.Aging (Albany NY). 2011 May;3(5):509-14. doi: 10.18632/aging.100324.
272 A small molecule TrkB ligand reduces motor impairment and neuropathology in R6/2 and BACHD mouse models of Huntington's disease.J Neurosci. 2013 Nov 27;33(48):18712-27. doi: 10.1523/JNEUROSCI.1310-13.2013.
273 Reduced pro-inflammatory responses to Staphylococcus aureus bloodstream infection and low prevalence of enterotoxin genes in isolates from patients on haemodialysis.Eur J Clin Microbiol Infect Dis. 2017 Jan;36(1):33-42. doi: 10.1007/s10096-016-2767-9. Epub 2016 Sep 9.
274 Transcriptomics of maternal and fetal membranes can discriminate between gestational-age matched preterm neonates with and without cognitive impairment diagnosed at 18-24 months.PLoS One. 2015 Mar 30;10(3):e0118573. doi: 10.1371/journal.pone.0118573. eCollection 2015.
275 HIPK3 modulates autophagy and HTT protein levels in neuronal and mouse models of Huntington disease.Autophagy. 2018;14(1):169-170. doi: 10.1080/15548627.2017.1393130. Epub 2018 Jan 29.
276 Inhibition of lipid signaling enzyme diacylglycerol kinase epsilon attenuates mutant huntingtin toxicity.J Biol Chem. 2012 Jun 15;287(25):21204-13. doi: 10.1074/jbc.M111.321661. Epub 2012 Apr 16.
277 Aggregation of scaffolding protein DISC1 dysregulates phosphodiesterase 4 in Huntington's disease.J Clin Invest. 2017 Apr 3;127(4):1438-1450. doi: 10.1172/JCI85594. Epub 2017 Mar 6.
278 SAP97-mediated rescue of NMDA receptor surface distribution in a neuronal model of Huntington's disease.Hippocampus. 2018 Oct;28(10):707-723. doi: 10.1002/hipo.22995.
279 Role of Dynein Axonemal Heavy Chain 6 Gene Expression as a Possible Biomarker for Huntington's Disease: a Translational Study.J Mol Neurosci. 2017 Dec;63(3-4):342-348. doi: 10.1007/s12031-017-0984-z. Epub 2017 Oct 10.
280 Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease.Proc Natl Acad Sci U S A. 2000 Mar 14;97(6):2898-903. doi: 10.1073/pnas.97.6.2898.
281 Suppression of protein aggregation by chaperone modification of high molecular weight complexes.Brain. 2012 Apr;135(Pt 4):1180-96. doi: 10.1093/brain/aws022. Epub 2012 Mar 6.
282 Astrocytic expression of the chaperone DNAJB6 results in non-cell autonomous protection in Huntington's disease.Neurobiol Dis. 2019 Apr;124:108-117. doi: 10.1016/j.nbd.2018.10.017. Epub 2018 Nov 5.
283 Interdigitated deletion complexes on mouse chromosome 5 induced by irradiation of embryonic stem cells.Genome Res. 2000 Jul;10(7):1043-50. doi: 10.1101/gr.10.7.1043.
284 DICER and DROSHA gene expression and polymorphisms in autoimmune thyroid diseases.Autoimmunity. 2016 Dec;49(8):514-522. doi: 10.1080/08916934.2016.1230846. Epub 2016 Nov 3.
285 Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity.J Neurosci. 2008 Sep 3;28(36):9013-20. doi: 10.1523/JNEUROSCI.2747-08.2008.
286 Early epigenomic and transcriptional changes reveal Elk-1 transcription factor as a therapeutic target in Huntington's disease.Proc Natl Acad Sci U S A. 2019 Dec 3;116(49):24840-24851. doi: 10.1073/pnas.1908113116. Epub 2019 Nov 19.
287 Antibiotic Prophylaxis for Melioidosis in Patients Receiving Hemodialysis in the Tropics? One Size Does Not Fit All.Am J Trop Med Hyg. 2018 Sep;99(3):597-600. doi: 10.4269/ajtmh.18-0421. Epub 2018 Jul 12.
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290 Neurological disease-associated autoantibodies against an unknown protein encoded by a RES4-22 homologous gene.Scand J Immunol. 2001 Feb;53(2):204-8. doi: 10.1046/j.1365-3083.2001.00839.x.
291 Variation in DNA Repair System Gene as an Additional Modifier of Age at Onset in Spinocerebellar Ataxia Type 3/Machado-Joseph Disease.Neuromolecular Med. 2020 Mar;22(1):133-138. doi: 10.1007/s12017-019-08572-4. Epub 2019 Oct 5.
292 Nuclease-deficient FEN-1 blocks Rad51/BRCA1-mediated repair and causes trinucleotide repeat instability.Mol Cell Biol. 2003 Sep;23(17):6063-74. doi: 10.1128/MCB.23.17.6063-6074.2003.
293 Drp1/Fis1-mediated mitochondrial fragmentation leads to lysosomal dysfunction in cardiac models of Huntington's disease.J Mol Cell Cardiol. 2019 Feb;127:125-133. doi: 10.1016/j.yjmcc.2018.12.004. Epub 2018 Dec 11.
294 NMR Spectroscopy-based Metabolomics of Drosophila Model of Huntington's Disease Suggests Altered Cell Energetics.J Proteome Res. 2017 Oct 6;16(10):3863-3872. doi: 10.1021/acs.jproteome.7b00491. Epub 2017 Sep 26.
295 FOXOs modulate proteasome activity in human-induced pluripotent stem cells of Huntington's disease and their derived neural cells.Hum Mol Genet. 2017 Nov 15;26(22):4416-4428. doi: 10.1093/hmg/ddx327.
296 Revealing Clusters of Connected Pathways Through Multisource Data Integration in Huntington's Disease and Spastic Ataxia.IEEE J Biomed Health Inform. 2019 Jan;23(1):26-37. doi: 10.1109/JBHI.2018.2865569. Epub 2018 Aug 30.
297 A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease.Mol Cell. 2004 Sep 24;15(6):853-65. doi: 10.1016/j.molcel.2004.09.016.
298 RNAi-Based GluN3A Silencing Prevents and Reverses Disease Phenotypes Induced by Mutant huntingtin.Mol Ther. 2018 Aug 1;26(8):1965-1972. doi: 10.1016/j.ymthe.2018.05.013. Epub 2018 Jun 15.
299 Altered hypothalamic protein expression in a rat model of Huntington's disease.PLoS One. 2012;7(10):e47240. doi: 10.1371/journal.pone.0047240. Epub 2012 Oct 18.
300 DNA damage signatures in peripheral blood cells as biomarkers in prodromal huntington disease.Ann Neurol. 2019 Feb;85(2):296-301. doi: 10.1002/ana.25393. Epub 2019 Jan 13.
301 Indexing disease progression at study entry with individuals at-risk for Huntington disease.Am J Med Genet B Neuropsychiatr Genet. 2011 Dec;156B(7):751-63. doi: 10.1002/ajmg.b.31232. Epub 2011 Aug 19.
302 HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.Hum Mol Genet. 2018 Jan 15;27(2):239-253. doi: 10.1093/hmg/ddx394.
303 Replacement of charged and polar residues in the coiled-coiled interface of huntingtin-interacting protein 1 (HIP1) causes aggregation and cell death.FEBS Lett. 2012 Sep 21;586(19):3030-6. doi: 10.1016/j.febslet.2012.07.011. Epub 2012 Jul 23.
304 Relationship of serum haemojuvelin and hepcidin levels with iron level and erythropoietin requirement in prevalent hepatitis C virus positive haemodialysis patients.Nephrology (Carlton). 2018 Apr;23(4):323-330. doi: 10.1111/nep.13010.
305 Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.J Neurochem. 2009 Oct;111(2):460-72. doi: 10.1111/j.1471-4159.2009.06344.x. Epub 2009 Aug 17.
306 Identification of genes associated with the effect of inflammation on the neurotransmission of vascular smooth muscle cell.Exp Ther Med. 2017 Apr;13(4):1303-1312. doi: 10.3892/etm.2017.4138. Epub 2017 Feb 21.
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308 Omi / HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease.Eur J Neurosci. 2008 Jul;28(1):30-40. doi: 10.1111/j.1460-9568.2008.06323.x.
309 Chaperone-like protein HYPK and its interacting partners augment autophagy.Eur J Cell Biol. 2016 Jun-Jul;95(6-7):182-94. doi: 10.1016/j.ejcb.2016.03.003. Epub 2016 Apr 1.
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313 Increased Act1/IL-17R expression in Hirschsprung's disease.Pediatr Surg Int. 2016 Dec;32(12):1201-1207. doi: 10.1007/s00383-016-3980-4. Epub 2016 Sep 22.
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317 Targeting the neuronal calcium sensor DREAM with small-molecules for Huntington's disease treatment.Sci Rep. 2019 May 13;9(1):7260. doi: 10.1038/s41598-019-43677-7.
318 HDAC inhibition imparts beneficial transgenerational effects in Huntington's disease mice via altered DNA and histone methylation.Proc Natl Acad Sci U S A. 2015 Jan 6;112(1):E56-64. doi: 10.1073/pnas.1415195112. Epub 2014 Dec 22.
319 SNP-guided microRNA maps (MirMaps) of 16 common human disorders identify a clinically accessible therapy reversing transcriptional aberrations of nuclear import and inflammasome pathways.Cell Cycle. 2008 Nov 15;7(22):3564-76. doi: 10.4161/cc.7.22.7073. Epub 2008 Nov 26.
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324 Hsa-miR-34b is a plasma-stable microRNA that is elevated in pre-manifest Huntington's disease.Hum Mol Genet. 2011 Jun 1;20(11):2225-37. doi: 10.1093/hmg/ddr111. Epub 2011 Mar 19.
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329 Receptor for Advanced Glycation End Products (RAGE) is Expressed Predominantly in Medium Spiny Neurons of tgHD Rat Striatum.Neuroscience. 2018 Jun 1;380:146-151. doi: 10.1016/j.neuroscience.2018.03.042. Epub 2018 Apr 4.
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336 Plasma neurofilament heavy chain levels in Huntington's disease.Neurosci Lett. 2007 May 7;417(3):231-3. doi: 10.1016/j.neulet.2007.02.053. Epub 2007 Feb 24.
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359 Adenyl cyclase activator forskolin protects against Huntington's disease-like neurodegenerative disorders.Neural Regen Res. 2017 Feb;12(2):290-300. doi: 10.4103/1673-5374.200812.
360 Protein C and protein S deficiencies may be related to survival among hemodialysis patients.BMC Nephrol. 2019 May 28;20(1):191. doi: 10.1186/s12882-019-1344-8.
361 Subjective Assessment of Sleep in Huntington Disease: Reliability of Sleep Questionnaires Compared to Polysomnography.Neurodegener Dis. 2017;17(6):330-337. doi: 10.1159/000480701. Epub 2017 Nov 24.
362 Gene therapy by proteasome activator, PA28, improves motor coordination and proteasome function in Huntington's disease YAC128 mice.Neuroscience. 2016 Jun 2;324:20-8. doi: 10.1016/j.neuroscience.2016.02.054. Epub 2016 Mar 2.
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364 Pharmacogenetic modulation of STEP improves motor and cognitive function in a mouse model of Huntington's disease.Neurobiol Dis. 2018 Dec;120:88-97. doi: 10.1016/j.nbd.2018.08.024. Epub 2018 Aug 31.
365 Striatal Vulnerability in Huntington's Disease: Neuroprotection Versus Neurotoxicity.Brain Sci. 2017 Jun 7;7(6):63. doi: 10.3390/brainsci7060063.
366 Glucose transporter 3 is a rab11-dependent trafficking cargo and its transport to the cell surface is reduced in neurons of CAG140 Huntington's disease mice.Acta Neuropathol Commun. 2014 Dec 20;2:179. doi: 10.1186/s40478-014-0178-7.
367 RAN Translation in Huntington Disease.Neuron. 2015 Nov 18;88(4):667-77. doi: 10.1016/j.neuron.2015.10.038.
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371 Serelaxin treatment promotes adaptive hypertrophy but does not prevent heart failure in experimental peripartum cardiomyopathy.Cardiovasc Res. 2017 May 1;113(6):598-608. doi: 10.1093/cvr/cvw245.
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379 SORLA-mediated trafficking of TrkB enhances the response of neurons to BDNF.PLoS One. 2013 Aug 19;8(8):e72164. doi: 10.1371/journal.pone.0072164. eCollection 2013.
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383 Pathophysiology in the suprachiasmatic nucleus in mouse models of Huntington's disease.J Neurosci Res. 2018 Dec;96(12):1862-1875. doi: 10.1002/jnr.24320. Epub 2018 Aug 31.
384 Preimplantation genetic diagnosis of P450 oxidoreductase deficiency and Huntington Disease using three different molecular approaches simultaneously.J Assist Reprod Genet. 2009 May;26(5):263-71. doi: 10.1007/s10815-009-9327-5. Epub 2009 Jul 21.
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387 SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation.Cell Rep. 2013 Jul 25;4(2):362-75. doi: 10.1016/j.celrep.2013.06.034. Epub 2013 Jul 18.
388 Effects on murine behavior and lifespan of selectively decreasing expression of mutant huntingtin allele by supt4h knockdown.PLoS Genet. 2015 Mar 11;11(3):e1005043. doi: 10.1371/journal.pgen.1005043. eCollection 2015 Mar.
389 Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms.J Cell Biol. 2013 Sep 30;202(7):1123-38. doi: 10.1083/jcb.201303146.
390 Huntington Mice Demonstrate Diminished Pain Response in Inflammatory Pain Model.Anesth Analg. 2018 Feb;126(2):661-669. doi: 10.1213/ANE.0000000000002419.
391 Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease.Science. 2002 Jun 21;296(5576):2238-43. doi: 10.1126/science.1072613. Epub 2002 May 2.
392 Metastasis-suppressor transcript destabilization through TARBP2 binding of mRNA hairpins.Nature. 2014 Sep 11;513(7517):256-60. doi: 10.1038/nature13466. Epub 2014 Jul 9.
393 Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23.Proc Natl Acad Sci U S A. 2019 Aug 13;116(33):16593-16602. doi: 10.1073/pnas.1904101116. Epub 2019 Jul 25.
394 p35 hemizygosity activates Akt but does not improve motor function in the YAC128 mouse model of Huntington's disease.Neuroscience. 2017 Jun 3;352:79-87. doi: 10.1016/j.neuroscience.2017.03.051. Epub 2017 Apr 6.
395 CAG Repeat Not Polyglutamine Length Determines Timing of Huntington's Disease Onset.Cell. 2019 Aug 8;178(4):887-900.e14. doi: 10.1016/j.cell.2019.06.036.
396 Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice.J Neurol Sci. 2008 Jan 15;264(1-2):77-86. doi: 10.1016/j.jns.2007.07.027. Epub 2007 Sep 12.