General Information of Disease (ID: DISDJJWE)

Disease Name Cholestasis
Synonyms obstruction of bile duct; bile occlusion
Definition Impairment of the bile flow caused by obstruction within the liver, or outside the liver in the bile duct system.
Disease Hierarchy
DISPFUV2: Bile duct disease
DISDJJWE: Cholestasis
Disease Identifiers
MONDO ID
MONDO_0001751
MESH ID
D002779
UMLS CUI
C0008370
MedGen ID
925
HPO ID
HP:0001396
SNOMED CT ID
197446008

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 88 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
USP53 OTQTAFCR Strong Autosomal recessive [1]
BCAP31 OTKSACR4 Limited Biomarker [65]
BEX4 OT3LXD15 Limited Biomarker [2]
BTG3 OT9ANHVT Limited Biomarker [2]
C4orf19 OTJMU7N6 Limited Biomarker [2]
CFHR5 OT7BMOYE Limited Biomarker [2]
GGTLC1 OTWJKUHQ Limited Biomarker [79]
GTPBP2 OTOJ1KUA Limited Biomarker [2]
HAO2 OTDWSQ0L Limited Biomarker [2]
HJV OT4235J2 Limited Biomarker [2]
HOGA1 OT7XETW4 Limited Biomarker [2]
ID4 OTPMJ39I Limited Biomarker [2]
KANK1 OT2E7A6W Limited Biomarker [2]
MBOAT1 OT4XR6TE Limited Biomarker [2]
NIBAN1 OTYOLI12 Limited Biomarker [2]
NPL OTA7P0TO Limited Biomarker [2]
RFLNB OTI7ZM75 Limited Biomarker [2]
SERPINA5 OTTZXPGD Limited Biomarker [2]
RDX OTNSYUN6 Disputed Altered Expression [80]
BCS1L OT5PY5CY moderate Biomarker [81]
GNAS OTMH8BKJ moderate Biomarker [82]
KLHL1 OTAX6SAD moderate Biomarker [83]
NLN OTFRITPU moderate Biomarker [84]
OPN4 OT1LZ7TS moderate Biomarker [84]
SIT1 OT7MDF09 moderate Biomarker [85]
SYCE1L OTXU44F3 moderate Biomarker [83]
AANAT OTUBJ7SX Strong Altered Expression [86]
ABCG8 OTIJ76XW Strong Biomarker [15]
ALDH1L1 OT15HOJX Strong Therapeutic [87]
AMFR OTQRX7LC Strong Posttranslational Modification [80]
AQP2 OTQLBKK6 Strong Biomarker [88]
AQP8 OT99JKME Strong Biomarker [89]
ASPG OT5E2EKR Strong Biomarker [90]
ATP8A1 OT2B7XBT Strong Altered Expression [91]
ATRNL1 OTY5JUX2 Strong Biomarker [92]
C1QTNF1 OT7I7KHC Strong Biomarker [93]
CADPS2 OTV1FW0M Strong Biomarker [66]
CCL7 OTDIS99H Strong Biomarker [25]
CELA3B OTGU8BE9 Strong Altered Expression [94]
CLOCK OTNEOJY7 Strong Biomarker [95]
CLTA OTLHOXMQ Strong Biomarker [37]
COL1A1 OTI31178 Strong Biomarker [96]
CYGB OTX153DQ Strong Biomarker [97]
DGUOK OT78HUZB Strong Genetic Variation [98]
DLAT OT9LBJVN Strong Biomarker [99]
DNAJB9 OT38EQT6 Strong Biomarker [66]
DNAJC12 OTNU59PT Strong Biomarker [66]
ERLEC1 OTQO3Z0R Strong Biomarker [66]
FAH OTGZA1YR Strong Biomarker [100]
FBL OTRODIE5 Strong Biomarker [101]
FGA OTMIHY80 Strong Biomarker [31]
GNMT OT0O2OQO Strong Biomarker [102]
GOLM1 OTOZSV6O Strong Biomarker [101]
HACL1 OT0XBOVJ Strong Biomarker [66]
HNF1B OTSYIC3T Strong Genetic Variation [103]
KIAA0586 OTOZRC1U Strong Genetic Variation [104]
MAF OT1GR3IZ Strong Altered Expression [76]
MAFG OTBQFUZH Strong Altered Expression [105]
MAPK13 OT0W9GE7 Strong Biomarker [46]
MAX OTKZ0YKM Strong Altered Expression [76]
MINDY4 OTBZ2SZB Strong Altered Expression [19]
MPV17 OT579DMU Strong Genetic Variation [98]
MYO5B OTCKL3W3 Strong Genetic Variation [106]
NFXL1 OT2Y5D15 Strong Biomarker [66]
ONECUT1 OTK1QUQT Strong Biomarker [107]
PAN2 OTB65N2I Strong Biomarker [66]
PCTP OTM36JXE Strong Altered Expression [108]
PDLIM3 OTVXQC81 Strong Biomarker [92]
PDYN OTEJ6430 Strong Biomarker [109]
PEX1 OTQJF0V7 Strong Genetic Variation [110]
PKHD1 OTAH8SMF Strong Genetic Variation [111]
PRKAA1 OT7TNF0L Strong Altered Expression [112]
RAB11A OTC4FW0J Strong Biomarker [113]
REEP5 OTZU4TJI Strong Biomarker [66]
RELA OTUJP9CN Strong Biomarker [46]
SCYL1 OTQ0IN7P Strong Genetic Variation [114]
SNAI1 OTDPYAMC Strong Biomarker [25]
SORL1 OTQ8FFNS Strong Biomarker [66]
SRP72 OTPV73W7 Strong Biomarker [66]
TAPBP OTL81AVZ Strong Biomarker [115]
TIMP1 OTOXC51H Strong Biomarker [116]
TJP1 OTBDCUPK Strong Biomarker [117]
TJP2 OTQUY6BV Strong Genetic Variation [118]
TMED7 OTONO8E6 Strong Biomarker [66]
TMEM117 OT4AV8FB Strong Biomarker [66]
HSD3B7 OT2Y9IW4 Definitive Biomarker [119]
KIF12 OTTALNDD Definitive Autosomal recessive [118]
TFAM OTXXV5V7 Definitive Altered Expression [120]
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⏷ Show the Full List of 88 DOT(s)
This Disease Is Related to 75 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
AGT TT5C0UB Limited Biomarker [2]
BCAT1 TTES57P Limited Biomarker [2]
DGAT2 TTRHEQ4 Limited Biomarker [2]
EGR1 TTE8LGD Limited Biomarker [3]
GGT1 TTZVT7O Limited Biomarker [4]
LY96 TT8S9AV Limited Biomarker [2]
PON1 TT9LX82 Limited Biomarker [2]
SERPINA1 TTA7UJC Limited Genetic Variation [5]
SLC23A1 TT6XAGK Limited Biomarker [6]
SLCO3A1 TT5CE6L Limited Altered Expression [7]
STAT1 TTN7R6K Limited CausalMutation [8]
TLR2 TTY7ZHS Limited Biomarker [2]
SLC23A2 TTOP832 Disputed Altered Expression [9]
ABCC4 TTUEAFL moderate Altered Expression [10]
CHIT1 TTDYX6T moderate Biomarker [11]
NR1I3 TTRANFM moderate Biomarker [12]
POMC TT21AKM moderate Genetic Variation [13]
ABCC3 TTVLG21 Strong Biomarker [14]
ABCG5 TTKZ7WY Strong Biomarker [15]
ADAMTS13 TTUREBK Strong Biomarker [16]
ADH1A TT5AHZ0 Strong Biomarker [17]
APOC3 TTXOZQ1 Strong Biomarker [18]
AQP1 TTSF1KH Strong Altered Expression [19]
ARG1 TT7ZQEV Strong Biomarker [20]
BLVRA TTJBPN3 Strong Biomarker [6]
CAT TTPS279 Strong Altered Expression [21]
CD14 TT6I7DC Strong Biomarker [22]
CD44 TTWFBT7 Strong Biomarker [23]
CFTR TTRLZHP Strong Biomarker [24]
CXCL2 TTZF0K2 Strong Biomarker [25]
CYP1A2 TTS1DTU Strong Biomarker [26]
CYP26A1 TTD7Q0R Strong Biomarker [27]
CYP2B6 TTMH124 Strong Biomarker [28]
CYP3A4 TTWP7HQ Strong Biomarker [29]
CYP3A5 TTHS0OK Strong Biomarker [28]
ENG TTB30LE Strong Biomarker [30]
F2RL3 TTD0652 Strong Biomarker [31]
FGF19 TTGCH11 Strong Biomarker [32]
G6PD TTKN8W0 Strong Genetic Variation [33]
GPBAR1 TTSDVTR Strong Biomarker [34]
GSTA1 TT4P8DE Strong Biomarker [35]
GSTP1 TT40K12 Strong Therapeutic [36]
GUCY2D TTWNFC2 Strong Biomarker [37]
HP TTLC8E1 Strong Biomarker [38]
ICAM1 TTA1L39 Strong Biomarker [25]
ID1 TTBXVDE Strong Biomarker [39]
IGF1 TTT6LOU Strong Biomarker [40]
IL1A TTPM6HI Strong Altered Expression [41]
IL4 TTLGTKB Strong Biomarker [42]
LAMP2 TTULDG7 Strong Biomarker [43]
LIPA TTS8T1M Strong Biomarker [44]
MAOB TTGP7BY Strong Biomarker [45]
MAPK14 TTQBR95 Strong Biomarker [46]
NOS2 TTF10I9 Strong Biomarker [46]
NQO1 TT8XK6L Strong Therapeutic [47]
NR1H2 TTXA6PH Strong Therapeutic [48]
NR1H3 TTECBXN Strong Therapeutic [48]
NR1I2 TT7LCTF Strong Altered Expression [49]
OTC TT5KIO9 Strong Biomarker [20]
PDGFB TTQA6SX Strong Biomarker [50]
PRDX4 TTPBL9I Strong Altered Expression [51]
PTBP1 TTWMX0U Strong Altered Expression [52]
PYGM TTZHY6R Strong Biomarker [53]
RHBDF2 TTH1ZOP Strong Biomarker [54]
S1PR2 TTVSMOH Strong Altered Expression [55]
SERPINE1 TTTO43N Strong Biomarker [25]
SLC10A1 TTWZRY5 Strong Therapeutic [56]
SLC10A2 TTPI1M5 Strong Biomarker [57]
SLC17A5 TTFSUIA Strong Altered Expression [58]
SLCO1B3 TTU86P0 Strong Biomarker [59]
SORT1 TTRX9AV Strong Biomarker [60]
TRPC5 TT32NQ1 Strong Biomarker [61]
VCAM1 TTHCEF6 Strong Biomarker [25]
XDH TT7RJY8 Strong Biomarker [62]
NFE2L2 TTA6ZN2 Definitive Biomarker [63]
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⏷ Show the Full List of 75 DTT(s)
This Disease Is Related to 9 DTP Molecule(s)
Gene Name DTP ID Evidence Level Mode of Inheritance REF
SLC16A2 DTQ8MP1 Limited Biomarker [2]
SLC30A10 DTYBI73 Limited Biomarker [2]
SLC51A DTMEQ32 Limited Biomarker [64]
ABCD1 DTKM9DZ Strong Genetic Variation [65]
SLC10A7 DTG1RJO Strong Biomarker [66]
SLC22A7 DT0OC1Q Strong Biomarker [67]
SLC25A13 DTDSYAQ Strong Genetic Variation [68]
SLC4A2 DTF7GAL Strong Altered Expression [69]
SLC51B DT1V9AJ Strong Biomarker [70]
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⏷ Show the Full List of 9 DTP(s)
This Disease Is Related to 20 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
ALDH1B1 DEXI4UQ Limited Biomarker [2]
ALDH8A1 DE870HF Limited Biomarker [2]
UGT1A3 DEF2WXN Limited Biomarker [71]
CYP8B1 DETL4WB Disputed Biomarker [72]
CYP27A1 DEBS639 moderate Altered Expression [72]
ADH5 DEIOH6A Strong Biomarker [73]
BAAT DERA3OF Strong Biomarker [74]
CYP2C18 DEZMWRE Strong Altered Expression [12]
CYP7A1 DEDZRQ1 Strong Therapeutic [56]
CYP7B1 DE36TMY Strong Biomarker [75]
GSTM1 DEYZEJA Strong Biomarker [35]
MAT1A DEQ6NC9 Strong Altered Expression [76]
SULT2A1 DE0P6LK Strong Biomarker [35]
SULT2B1 DEZBN53 Strong Biomarker [77]
UGT1A10 DEL5N6Y Strong Altered Expression [78]
UGT1A4 DELOY3P Strong Altered Expression [78]
UGT1A6 DESD26P Strong Altered Expression [78]
UGT1A7 DEZO4N3 Strong Altered Expression [78]
UGT1A8 DE2GB8N Strong Altered Expression [78]
UGT2B4 DENUPDX Strong Biomarker [35]
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⏷ Show the Full List of 20 DME(s)

References

1 Classification of Genes: Standardized Clinical Validity Assessment of Gene-Disease Associations Aids Diagnostic Exome Analysis and Reclassifications. Hum Mutat. 2017 May;38(5):600-608. doi: 10.1002/humu.23183. Epub 2017 Feb 13.
2 Integrative "-Omics" analysis in primary human hepatocytes unravels persistent mechanisms of cyclosporine A-induced cholestasis. Chem Res Toxicol. 2016 Dec 19;29(12):2164-2174.
3 Anti-inflammatory, anti-oxidative stress and novel therapeutic targets for cholestatic liver injury.Biosci Trends. 2019 Mar 14;13(1):23-31. doi: 10.5582/bst.2018.01247. Epub 2019 Feb 27.
4 Serum bile acids in patients with hepatopulmonary syndrome.Z Gastroenterol. 2017 Apr;55(4):361-367. doi: 10.1055/s-0042-121268. Epub 2016 Dec 12.
5 Analysis of gene mutations in children with cholestasis of undefined etiology.J Pediatr Gastroenterol Nutr. 2010 Oct;51(4):488-493. doi: 10.1097/MPG.0b013e3181dffe8f.
6 Role of vitamin C transporters and biliverdin reductase in the dual pro-oxidant and anti-oxidant effect of biliary compounds on the placental-fetal unit in cholestasis during pregnancy. Toxicol Appl Pharmacol. 2008 Oct 15;232(2):327-36.
7 Solute Carrier Organic Anion Transporter Family Member 3A1 Is a Bile Acid Efflux Transporter in Cholestasis.Gastroenterology. 2018 Nov;155(5):1578-1592.e16. doi: 10.1053/j.gastro.2018.07.031. Epub 2018 Jul 29.
8 A novel presentation of homozygous loss-of-function STAT-1 mutation in an infant with hyperinflammation-A case report and review of the literature.J Allergy Clin Immunol Pract. 2016 Jul-Aug;4(4):777-9. doi: 10.1016/j.jaip.2016.02.015. Epub 2016 Apr 23.
9 Liver metabolic/oxidative stress induces hepatic and extrahepatic changes in the expression of the vitamin C transporters SVCT1 and SVCT2.Eur J Nutr. 2014;53(2):401-12. doi: 10.1007/s00394-013-0536-4. Epub 2013 May 26.
10 TNF Induces Multidrug Resistance-Associated Protein 4 Expression through p38-E2F1-Nrf2 Signaling in Obstructive Cholestasis.Yonsei Med J. 2019 Nov;60(11):1045-1053. doi: 10.3349/ymj.2019.60.11.1045.
11 Oxysterol/chitotriosidase based selective screening for Niemann-Pick type C in infantile cholestasis syndrome patients.BMC Med Genet. 2019 Jul 11;20(1):123. doi: 10.1186/s12881-019-0857-0.
12 Xenobiotic Nuclear Receptor Signaling Determines Molecular Pathogenesis of Progressive Familial Intrahepatic Cholestasis.Endocrinology. 2018 Jun 1;159(6):2435-2446. doi: 10.1210/en.2018-00110.
13 A Nonsense Mutation in the Hedgehog Receptor CDON Associated With Pituitary Stalk Interruption Syndrome. J Clin Endocrinol Metab. 2016 Jan;101(1):12-5. doi: 10.1210/jc.2015-2995. Epub 2015 Nov 3.
14 9-cis-retinoic acid elevates MRP3 expression by inhibiting sumoylation of RXR to alleviate cholestatic liver injury.Biochem Biophys Res Commun. 2018 Sep 3;503(1):188-194. doi: 10.1016/j.bbrc.2018.06.001. Epub 2018 Jun 18.
15 Effect of maternal cholestasis and treatment with ursodeoxycholic acid on the expression of genes involved in the secretion of biliary lipids by the neonatal rat liver.Life Sci. 2006 Aug 1;79(10):1014-9. doi: 10.1016/j.lfs.2006.05.012. Epub 2006 May 20.
16 Increased production of ADAMTS13 in hepatic stellate cells contributes to enhanced plasma ADAMTS13 activity in rat models of cholestasis and steatohepatitis.Thromb Haemost. 2009 Aug;102(2):389-96. doi: 10.1160/TH08-11-0732.
17 Identification of Serum Biomarkers to Distinguish Hazardous and Benign Aminotransferase Elevations.Toxicol Sci. 2020 Feb 1;173(2):244-254. doi: 10.1093/toxsci/kfz222.
18 Effect of bile duct obstruction on the expression of intestinal mRNA related to cholesterol and bile acid metabolism in the rat.J Gastroenterol Hepatol. 2007 Jan;22(1):125-31. doi: 10.1111/j.1440-1746.2006.04365.x.
19 Improved hepatic MRP2/ABCC2 transport activity in LPS-induced cholestasis by aquaporin-1 gene transfer.Biochimie. 2019 Oct;165:179-182. doi: 10.1016/j.biochi.2019.07.027. Epub 2019 Aug 1.
20 The activity of ornithine transcarbamoylase and arginase during mechanical jaundice in the rat model.J Surg Res. 2005 Jun 1;126(1):19-26. doi: 10.1016/j.jss.2005.01.026.
21 Comparison between the effects of selenomethionine and S-adenosylmethionine in preventing cholestasis-induced rat liver damage.Amino Acids. 2019 May;51(5):795-803. doi: 10.1007/s00726-019-02716-3. Epub 2019 Mar 16.
22 Effects of hepatocyte CD14 upregulation during cholestasis on endotoxin sensitivity.PLoS One. 2012;7(4):e34903. doi: 10.1371/journal.pone.0034903. Epub 2012 Apr 12.
23 Effect of vitamin A on the CD44 expression in the small intestine of rats with obstructive jaundice.Eur Surg Res. 2006;38(3):347-52. doi: 10.1159/000094148. Epub 2006 Jun 23.
24 Increased expression of cystic fibrosis transmembrane conductance regulator in rat liver after common bile duct ligation.J Cell Physiol. 2005 Jun;203(3):599-603. doi: 10.1002/jcp.20259.
25 Bile acids induce inflammatory genes in hepatocytes: a novel mechanism of inflammation during obstructive cholestasis.Am J Pathol. 2011 Jan;178(1):175-86. doi: 10.1016/j.ajpath.2010.11.026. Epub 2010 Dec 23.
26 The impact of serotonergic system dysfunction on the regulation of P4501A isoforms during liver insufficiency and consequences for thyroid hormone homeostasis.Food Chem Toxicol. 2016 Nov;97:70-81. doi: 10.1016/j.fct.2016.08.027. Epub 2016 Aug 24.
27 Altered Expression of Retinol Metabolism-Related Genes in an ANIT-Induced Cholestasis Rat Model.Int J Mol Sci. 2018 Oct 26;19(11):3337. doi: 10.3390/ijms19113337.
28 Organic anion transporting polypeptide 1a1 null mice are sensitive to cholestatic liver injury.Toxicol Sci. 2012 Jun;127(2):451-62. doi: 10.1093/toxsci/kfs123. Epub 2012 Mar 29.
29 Pregnane X receptor and constitutive androstane receptor modulate differently CYP3A-mediated metabolism in early- and late-stage cholestasis.World J Gastroenterol. 2017 Nov 14;23(42):7519-7530. doi: 10.3748/wjg.v23.i42.7519.
30 Expression and functional analysis of endoglin in isolated liver cells and its involvement in fibrogenic Smad signalling.Cell Signal. 2011 Apr;23(4):683-99. doi: 10.1016/j.cellsig.2010.12.002. Epub 2010 Dec 10.
31 Role of fibrinogen and protease-activated receptors in acute xenobiotic-induced cholestatic liver injury.Toxicol Sci. 2011 Jan;119(1):233-43. doi: 10.1093/toxsci/kfq327. Epub 2010 Oct 25.
32 Fibroblast growth factors in control of lipid metabolism: from biological function to clinical application.Curr Opin Lipidol. 2019 Jun;30(3):235-243. doi: 10.1097/MOL.0000000000000599.
33 Cooperating G6PD mutations associated with severe neonatal hyperbilirubinemia and cholestasis.Pediatr Blood Cancer. 2011 May;56(5):840-2. doi: 10.1002/pbc.22744. Epub 2010 Oct 14.
34 Bile acids increase steroidogenesis in cholemic mice and induce cortisol secretion in adrenocortical H295R cells via S1PR2, ERK and SF-1.Liver Int. 2019 Nov;39(11):2112-2123. doi: 10.1111/liv.14052. Epub 2019 Feb 17.
35 Hepatic expression of detoxification enzymes is decreased in human obstructive cholestasis due to gallstone biliary obstruction.PLoS One. 2015 Mar 23;10(3):e0120055. doi: 10.1371/journal.pone.0120055. eCollection 2015.
36 Protective effect of Jasonia montana against ethinylestradiol-induced cholestasis in rats.Saudi Pharm J. 2010 Jan;18(1):27-33. doi: 10.1016/j.jsps.2009.12.002. Epub 2009 Dec 23.
37 Tanshinone IIA exerts protective effects in a LCA-induced cholestatic liver model associated with participation of pregnane X receptor.J Ethnopharmacol. 2015 Apr 22;164:357-67. doi: 10.1016/j.jep.2015.01.047. Epub 2015 Feb 7.
38 Effect of glutathione depletion and hydrophilic bile acids on hepatic acute phase reaction in rats with extrahepatic cholestasis.Scand J Gastroenterol. 2003 Aug;38(8):878-85. doi: 10.1080/00365520310003471.
39 Id1 is a critical mediator in TGF-beta-induced transdifferentiation of rat hepatic stellate cells.Hepatology. 2006 May;43(5):1032-41. doi: 10.1002/hep.21135.
40 Insulin-like growth factor-1 isoforms in rat hepatocytes and cholangiocytes and their involvement in protection against cholestatic injury.Lab Invest. 2008 Sep;88(9):986-94. doi: 10.1038/labinvest.2008.63. Epub 2008 Jul 7.
41 Molecular network-based analysis of the mechanism of liver injury induced by volatile oils from Artemisiae argyi folium.BMC Complement Altern Med. 2017 Nov 16;17(1):491. doi: 10.1186/s12906-017-1997-4.
42 Splanchnic Th(2) and Th(1) cytokine redistribution in microsurgical cholestatic rats.J Surg Res. 2010 Aug;162(2):203-12. doi: 10.1016/j.jss.2009.06.010. Epub 2009 Nov 11.
43 A Critical Evaluation of Liver Pathology in Humans with Danon Disease and Experimental Correlates in a Rat Model of LAMP-2 Deficiency.Clin Rev Allergy Immunol. 2017 Aug;53(1):105-116. doi: 10.1007/s12016-017-8598-3.
44 A splice junction mutation causes deletion of a 72-base exon from the mRNA for lysosomal acid lipase in a patient with cholesteryl ester storage disease.J Clin Invest. 1993 Dec;92(6):2713-8. doi: 10.1172/JCI116888.
45 Metabolic changes in rat brain histaminergic neurons during subhepatic cholestasis.Neurosci Behav Physiol. 2008 Oct;38(8):807-10. doi: 10.1007/s11055-008-9052-z. Epub 2008 Sep 18.
46 Mechanisms of TNFalpha-induced cardiac dysfunction in cholestatic bile duct-ligated mice: interaction between TNFalpha and endocannabinoids.J Hepatol. 2010 Aug;53(2):298-306. doi: 10.1016/j.jhep.2010.03.011. Epub 2010 May 6.
47 Dioscin Protects ANIT-Induced Intrahepatic Cholestasis Through Regulating Transporters, Apoptosis and Oxidative Stress.Front Pharmacol. 2017 Mar 8;8:116. doi: 10.3389/fphar.2017.00116. eCollection 2017.
48 Activation of LXRs prevents bile acid toxicity and cholestasis in female mice. Hepatology. 2007 Feb;45(2):422-32. doi: 10.1002/hep.21494.
49 Lignans from Schisandra sphenanthera protect against lithocholic acid-induced cholestasis by pregnane X receptor activation in mice.J Ethnopharmacol. 2019 Dec 5;245:112103. doi: 10.1016/j.jep.2019.112103. Epub 2019 Jul 20.
50 Expression of platelet-derived growth factor in newly formed cholangiocytes during experimental biliary fibrosis in rats.J Hepatol. 1999 Jul;31(1):100-9. doi: 10.1016/s0168-8278(99)80169-x.
51 Protective Effects of Peroxiredoxin 4 (PRDX4) on Cholestatic Liver Injury.Int J Mol Sci. 2018 Aug 24;19(9):2509. doi: 10.3390/ijms19092509.
52 H19 potentiates let-7 family expression through reducing PTBP1 binding to their precursors in cholestasis.Cell Death Dis. 2019 Feb 18;10(3):168. doi: 10.1038/s41419-019-1423-6.
53 Hepatic and skeletal muscle glycogen metabolism in rats with short-term cholestasis.J Hepatol. 2002 Jan;36(1):22-9. doi: 10.1016/s0168-8278(01)00287-2.
54 iRhom2 inhibits bile duct obstruction-induced liver fibrosis.Sci Signal. 2019 Oct 29;12(605):eaax1194. doi: 10.1126/scisignal.aax1194.
55 The role of sphingosine 1-phosphate receptor 2 in bile-acid-induced cholangiocyte proliferation and cholestasis-induced liver injury in mice.Hepatology. 2017 Jun;65(6):2005-2018. doi: 10.1002/hep.29076. Epub 2017 Apr 28.
56 Alisma orientale extract exerts the reversing cholestasis effect by activation of farnesoid X receptor.Phytomedicine. 2018 Mar 15;42:34-42. doi: 10.1016/j.phymed.2018.03.017. Epub 2018 Mar 8.
57 Bile Acids in Cholestasis and its Treatment.Ann Hepatol. 2017 Nov;16(Suppl. 1: s3-105.):s53-s57. doi: 10.5604/01.3001.0010.5497.
58 Metabolomics and Lipidomics Reveal the Effect of Hepatic Vps33b Deficiency on Bile Acids and Lipids Metabolism.Front Pharmacol. 2019 Mar 22;10:276. doi: 10.3389/fphar.2019.00276. eCollection 2019.
59 Predicting Drug-Induced Cholestasis with the Help of Hepatic Transporters-An in Silico Modeling Approach.J Chem Inf Model. 2017 Mar 27;57(3):608-615. doi: 10.1021/acs.jcim.6b00518. Epub 2017 Mar 8.
60 Sortilin 1 Loss-of-Function Protects Against Cholestatic Liver Injury by Attenuating Hepatic Bile Acid Accumulation in Bile Duct Ligated Mice.Toxicol Sci. 2018 Jan 1;161(1):34-47. doi: 10.1093/toxsci/kfx078.
61 Transient receptor potential canonical 5 channels plays an essential role in hepatic dyslipidemia associated with cholestasis.Sci Rep. 2017 May 24;7(1):2338. doi: 10.1038/s41598-017-02439-z.
62 The impact of hepatic xanthine oxidase and xanthine dehydrogenase activities on liver function in chronic cholestasis.Pediatr Surg Int. 2000;16(4):297-301. doi: 10.1007/s003830050748.
63 Effects of Hepatic Ischemia-Reperfusion Injuries and NRF2 on Transcriptional Activities of Bile Transporters in Rats.J Surg Res. 2019 Mar;235:73-82. doi: 10.1016/j.jss.2018.09.057. Epub 2018 Oct 24.
64 Clinical application of transcriptional activators of bile salt transporters.Mol Aspects Med. 2014 Jun;37(100):57-76. doi: 10.1016/j.mam.2013.12.001. Epub 2013 Dec 12.
65 Genotype-phenotype correlation of contiguous gene deletions of SLC6A8, BCAP31 and ABCD1.Clin Genet. 2015 Feb;87(2):141-7. doi: 10.1111/cge.12355. Epub 2014 Mar 6.
66 Classification of Cholestatic and Necrotic Hepatotoxicants Using Transcriptomics on Human Precision-Cut Liver Slices.Chem Res Toxicol. 2016 Mar 21;29(3):342-51. doi: 10.1021/acs.chemrestox.5b00491. Epub 2016 Mar 9.
67 Nuclear receptors constitutive androstane receptor and pregnane X receptor ameliorate cholestatic liver injury.Proc Natl Acad Sci U S A. 2005 Feb 8;102(6):2063-8. doi: 10.1073/pnas.0409794102. Epub 2005 Jan 31.
68 Screening of SLC25A13 mutations in early and late onset patients with citrin deficiency and in the Japanese population: Identification of two novel mutations and establishment of multiple DNA diagnosis methods for nine mutations.Hum Mutat. 2002 Feb;19(2):122-30. doi: 10.1002/humu.10022.
69 Adaptive downregulation of Cl-/HCO3- exchange activity in rat hepatocytes under experimental obstructive cholestasis.PLoS One. 2019 Feb 21;14(2):e0212215. doi: 10.1371/journal.pone.0212215. eCollection 2019.
70 Organic solute transporter- (SLC51B) deficiency in two brothers with congenital diarrhea and features of cholestasis. Hepatology. 2018 Aug;68(2):590-598. doi: 10.1002/hep.29516. Epub 2018 May 11.
71 Regulation of the human bile acid UDP-glucuronosyltransferase 1A3 by the farnesoid X receptor and bile acids. J Hepatol. 2010 Apr;52(4):570-8.
72 The ileum-liver Farnesoid X Receptor signaling axis mediates the compensatory mechanism of 17-ethynylestradiol-induced cholestasis via increasing hepatic biosynthesis of chenodeoxycholic acids in rats.Eur J Pharm Sci. 2018 Oct 15;123:404-415. doi: 10.1016/j.ejps.2018.08.005. Epub 2018 Aug 2.
73 Inhibition of nitric oxide synthesis during induced cholestasis ameliorates hepatocellular injury by facilitating S-nitrosothiol homeostasis.Lab Invest. 2010 Jan;90(1):116-27. doi: 10.1038/labinvest.2009.104. Epub 2009 Oct 5.
74 Genetic defects in bile acid conjugation cause fat-soluble vitamin deficiency.Gastroenterology. 2013 May;144(5):945-955.e6; quiz e14-5. doi: 10.1053/j.gastro.2013.02.004. Epub 2013 Feb 13.
75 Identification of a new inborn error in bile acid synthesis: mutation of the oxysterol 7alpha-hydroxylase gene causes severe neonatal liver disease. J Clin Invest. 1998 Nov 1;102(9):1690-703. doi: 10.1172/JCI2962.
76 Prohibitin 1 suppresses liver cancer tumorigenesis in mice and human hepatocellular and cholangiocarcinoma cells.Hepatology. 2017 Apr;65(4):1249-1266. doi: 10.1002/hep.28964. Epub 2017 Jan 31.
77 Upregulation of hydroxysteroid sulfotransferase 2B1b promotes hepatic oval cell proliferation by modulating oxysterol-induced LXR activation in a mouse model of liver injury. Arch Toxicol. 2017 Jan;91(1):271-287. doi: 10.1007/s00204-016-1693-z. Epub 2016 Apr 6.
78 Yinchenhao Decoction Ameliorates Alpha-Naphthylisothiocyanate Induced Intrahepatic Cholestasis in Rats by Regulating Phase II Metabolic Enzymes and Transporters.Front Pharmacol. 2018 May 15;9:510. doi: 10.3389/fphar.2018.00510. eCollection 2018.
79 Hepatic Lesions Associated With McCune Albright Syndrome.J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):e54-e57. doi: 10.1097/MPG.0000000000002266.
80 Canalicular membrane MRP2/ABCC2 internalization is determined by Ezrin Thr567 phosphorylation in human obstructive cholestasis.J Hepatol. 2015 Dec;63(6):1440-8. doi: 10.1016/j.jhep.2015.07.016. Epub 2015 Jul 23.
81 GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Am J Hum Genet. 2002 Oct;71(4):863-76. doi: 10.1086/342773. Epub 2002 Sep 5.
82 Demonstration of McCune-Albright mutations in the liver of children with high gammaGT progressive cholestasis.J Hepatol. 2000 Jan;32(1):154-8. doi: 10.1016/s0168-8278(00)80202-0.
83 Hepatoprotection of auraptene from the peels of citrus fruits against 17-ethinylestradiol-induced cholestasis in mice by activating farnesoid X receptor.Food Funct. 2019 Jul 17;10(7):3839-3850. doi: 10.1039/c9fo00318e.
84 Adaptive homeostasis of the vitamin D-vitamin D nuclear receptor axis in 8-methoxypsoralen-induced hepatotoxicity. Toxicol Appl Pharmacol. 2019 Jan 1;362:150-158. doi: 10.1016/j.taap.2018.11.002. Epub 2018 Nov 10.
85 From the Cover: MechanisticInsights in Cytotoxic and Cholestatic Potential of the Endothelial Receptor Antagonists Using HepaRG Cells. Toxicol Sci. 2017 Jun 1;157(2):451-464. doi: 10.1093/toxsci/kfx062.
86 Pinealectomy or light exposure exacerbates biliary damage and liver fibrosis in cholestatic rats through decreased melatonin synthesis.Biochim Biophys Acta Mol Basis Dis. 2019 Jun 1;1865(6):1525-1539. doi: 10.1016/j.bbadis.2019.03.002. Epub 2019 Mar 16.
87 Exploration of Hepatoprotective Effect of Gentiopicroside on Alpha-Naphthylisothiocyanate-Induced Cholestatic Liver Injury in Rats by Comprehensive Proteomic and Metabolomic Signatures.Cell Physiol Biochem. 2018;49(4):1304-1319. doi: 10.1159/000493409. Epub 2018 Sep 17.
88 Cholemic Nephropathy Causes Acute Kidney Injury and Is Accompanied by Loss of Aquaporin 2 in Collecting Ducts.Hepatology. 2019 May;69(5):2107-2119. doi: 10.1002/hep.30499. Epub 2019 Mar 14.
89 Adenoviral transfer of human aquaporin-1 gene to rat liver improves bile flow in estrogen-induced cholestasis.Gene Ther. 2014 Dec;21(12):1058-64. doi: 10.1038/gt.2014.78. Epub 2014 Sep 11.
90 Asparaginase-induced hepatotoxicity: rapid development of cholestasis and hepatic steatosis.Hepatol Int. 2019 Sep;13(5):641-648. doi: 10.1007/s12072-019-09971-2. Epub 2019 Aug 7.
91 A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells.Hepatology. 2010 Jun;51(6):2049-60. doi: 10.1002/hep.23586.
92 Paeoniflorin attenuates ANIT-induced cholestasis by inhibiting apoptosis in vivo via mitochondria-dependent pathway.Biomed Pharmacother. 2017 May;89:696-704. doi: 10.1016/j.biopha.2017.02.084. Epub 2017 Mar 6.
93 Elevated CTRP1 Plasma Concentration Is Associated with Sepsis and Pre-Existing Type 2 Diabetes Mellitus in Critically Ill Patients.J Clin Med. 2019 May 11;8(5):661. doi: 10.3390/jcm8050661.
94 Fecal elastase 1, serum amylase and lipase levels in children with cholestasis.Pancreatology. 2005;5(4-5):432-7. doi: 10.1159/000086545. Epub 2005 Jun 28.
95 Melatonin inhibits cholangiocyte hyperplasia in cholestatic rats by interaction with MT1 but not MT2 melatonin receptors.Am J Physiol Gastrointest Liver Physiol. 2011 Oct;301(4):G634-43. doi: 10.1152/ajpgi.00206.2011. Epub 2011 Jul 14.
96 Aqueous extract of Artemisia iwayomogi Kitamura attenuates cholestatic liver fibrosis in a rat model of bile duct ligation.Food Chem Toxicol. 2012 Oct;50(10):3505-13. doi: 10.1016/j.fct.2012.07.018. Epub 2012 Jul 20.
97 Possible Involvement of Nitric Oxide in Enhanced Liver Injury and Fibrogenesis during Cholestasis in Cytoglobin-deficient Mice.Sci Rep. 2017 Feb 3;7:41888. doi: 10.1038/srep41888.
98 Clinical and molecular characteristics of mitochondrial DNA depletion syndrome associated with neonatal cholestasis and liver failure.J Pediatr. 2014 Mar;164(3):553-9.e1-2. doi: 10.1016/j.jpeds.2013.10.082. Epub 2013 Dec 8.
99 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines.Gut. 2018 Sep;67(9):1568-1594. doi: 10.1136/gutjnl-2017-315259. Epub 2018 Mar 28.
100 Tyrosinaemia type I--de novo mutation in liver tissue suppressing an inborn splicing defect.J Mol Med (Berl). 2005 May;83(5):406-10. doi: 10.1007/s00109-005-0648-2. Epub 2005 Mar 10.
101 The Clinical Significance of GP73 in Immunologically Mediated Chronic Liver Diseases: Experimental Data and Literature Review.Clin Rev Allergy Immunol. 2018 Apr;54(2):282-294. doi: 10.1007/s12016-017-8655-y.
102 MiR-873-5p acts as an epigenetic regulator in early stages of liver fibrosis and cirrhosis.Cell Death Dis. 2018 Sep 20;9(10):958. doi: 10.1038/s41419-018-1014-y.
103 Hepatic phenotypes of HNF1B gene mutations: a case of neonatal cholestasis requiring portoenterostomy and literature review.World J Gastroenterol. 2015 Feb 28;21(8):2550-7. doi: 10.3748/wjg.v21.i8.2550.
104 Loss of cilia causes embryonic lung hypoplasia, liver fibrosis, and cholestasis in the talpid3 ciliopathy mutant.Organogenesis. 2014 Apr-Jun;10(2):177-85. doi: 10.4161/org.28819. Epub 2014 Apr 17.
105 Mechanisms of MAFG Dysregulation in Cholestatic Liver Injury and Development of Liver Cancer.Gastroenterology. 2018 Aug;155(2):557-571.e14. doi: 10.1053/j.gastro.2018.04.032. Epub 2018 May 5.
106 MYO5B mutations cause cholestasis with normal serum gamma-glutamyl transferase activity in children without microvillous inclusion disease. Hepatology. 2017 Jan;65(1):164-173. doi: 10.1002/hep.28779. Epub 2016 Oct 5.
107 Pathophysiologic role of hepatocyte nuclear factor 6.Cell Signal. 2012 Jan;24(1):9-16. doi: 10.1016/j.cellsig.2011.08.009. Epub 2011 Aug 27.
108 Etiologic significance of defects in cholesterol, phospholipid, and bile acid metabolism in the liver of patients with intrahepatic calculi.Hepatology. 2001 May;33(5):1194-205. doi: 10.1053/jhep.2001.23936.
109 Nalfurafine, a kappa opioid receptor agonist, inhibits scratching behavior secondary to cholestasis induced by chronic ethynylestradiol injections in rats.Pharmacol Biochem Behav. 2006 Sep;85(1):39-43. doi: 10.1016/j.pbb.2006.07.004. Epub 2006 Aug 17.
110 The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder.Mol Genet Metab. 2014 Apr;111(4):522-532. doi: 10.1016/j.ymgme.2014.01.008. Epub 2014 Jan 23.
111 Taking the next step forward - Diagnosing inherited infantile cholestatic disorders with next generation sequencing.Mol Cell Probes. 2015 Oct;29(5):291-8. doi: 10.1016/j.mcp.2015.03.001. Epub 2015 Mar 13.
112 Role of AMP-activated protein kinase 1 in 17-ethinylestradiol-induced cholestasis in rats.Arch Toxicol. 2017 Jan;91(1):481-494. doi: 10.1007/s00204-016-1697-8. Epub 2016 Apr 18.
113 MYO5B and bile salt export pump contribute to cholestatic liver disorder in microvillous inclusion disease.Hepatology. 2014 Jul;60(1):301-10. doi: 10.1002/hep.26974. Epub 2014 May 27.
114 Generation of an induced pluripotent stem cell (iPSC) line, DHMCi005-A, from a patient with CALFAN syndrome due to mutations in SCYL1.Stem Cell Res. 2019 May;37:101428. doi: 10.1016/j.scr.2019.101428. Epub 2019 Mar 22.
115 Understanding the relationship between hospital volume and patient outcomes for infants with gastroschisis.J Pediatr Surg. 2017 Dec;52(12):1977-1980. doi: 10.1016/j.jpedsurg.2017.08.065. Epub 2017 Sep 5.
116 Tissue inhibitor of metalloproteinase-1 messenger RNA expression is enhanced relative to interstitial collagenase messenger RNA in experimental liver injury and fibrosis.Hepatology. 1996 Jul;24(1):176-84. doi: 10.1002/hep.510240129.
117 Bile duct ligation in the rat causes upregulation of ZO-2 and decreased colocalization of claudins with ZO-1 and occludin.Histochem Cell Biol. 2008 Mar;129(3):289-99. doi: 10.1007/s00418-007-0374-7. Epub 2008 Jan 15.
118 Identification of novel loci for pediatric cholestatic liver disease defined by KIF12, PPM1F, USP53, LSR, and WDR83OS pathogenic variants. Genet Med. 2019 May;21(5):1164-1172. doi: 10.1038/s41436-018-0288-x. Epub 2018 Sep 25.
119 Towards a systematic analysis of human short-chain dehydrogenases/reductases (SDR): Ligand identification and structure-activity relationships. Chem Biol Interact. 2015 Jun 5;234:114-25.
120 Effect of cholestasis and NeuroAid treatment on the expression of Bax, Bcl-2, Pgc-1 and Tfam genes involved in apoptosis and mitochondrial biogenesis in the striatum of male rats.Metab Brain Dis. 2020 Jan;35(1):183-192. doi: 10.1007/s11011-019-00508-y. Epub 2019 Nov 26.