Details of Disease

General Information of Disease (ID: DIS43E0O)

Disease Name	Primary biliary cholangitis
Synonyms	familial primary biliary cirrhosis; biliary cirrhosis, primary; Hanot syndrome; primary biliary cirrhosis; biliary liver cirrhosis; primary Bilary cirrhosis (PBC); PBC; chronic non-suppurative destructive cholangitis; chronic nonsuppurative destructive cholangitis; cholestatic cirrhosis
Disease Class	DB96: Autoimmune liver disease
Definition	Primary biliary cholangitis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure.
Disease Hierarchy	DISNBDRC: Biliary tract disorder DISGG52B: Cirrhosis, familial DIS43E0O: Primary biliary cholangitis
ICD Code	ICD-11 ICD-11: DB96.1 Expand ICD-11 'DB96.1 Expand ICD-10 'K74.3; 'K74.5; 'K74.6
Disease Identifiers	MONDO ID MONDO_0005388 MESH ID D008105 UMLS CUI C0008312 MedGen ID 3035 Orphanet ID 186 SNOMED CT ID 31712002

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
obeticholic acid	DM3Q1SM	Approved	Small molecular drug	[1]
Ursodeoxycholic acid	DMCUT21	Approved	Small molecular drug	[2]
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This Disease is Treated as An Indication in 4 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
MBX-8025	DMNTCV4	Phase 2/3	Small molecular drug	[3]
EDP-305	DMGJYZ8	Phase 2	Small molecular drug	[4]
LJN452	DM3XRUH	Phase 2	NA	[3]
LUM001	DMQZXDW	Phase 2	NA	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 78 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CD14	TT6I7DC	Limited	Altered Expression	[5]
CD19	TTW640A	Limited	Biomarker	[6]
DDR2	TTU98HG	Limited	Altered Expression	[7]
GGT1	TTZVT7O	Limited	Altered Expression	[8]
SLCO1B3	TTU86P0	Limited	Biomarker	[9]
TNFSF11	TT9E8HR	Limited	Biomarker	[10]
TNFSF15	TTEST6I	Limited	Biomarker	[11]
ACE2	TTUI5H7	Disputed	Biomarker	[12]
AGT	TT5C0UB	Disputed	Biomarker	[12]
CCN2	TTIL516	Disputed	Biomarker	[13]
DAG1	TT4X7PG	Disputed	Biomarker	[14]
JAK2	TTRMX3V	Disputed	Therapeutic	[15]
MAP3K14	TT4LIAC	Disputed	Biomarker	[16]
MAS1	TTOISYB	Disputed	Biomarker	[12]
MMP13	TTHY57M	Disputed	Therapeutic	[17]
MMP3	TTUZ2L5	Disputed	Therapeutic	[17]
PDE5A	TTJ0IQB	Disputed	Biomarker	[18]
REN	TTB2MXP	Disputed	Biomarker	[19]
RPS6KB1	TTG0U4H	Disputed	Biomarker	[20]
SERPINB3	TT6QLPX	Disputed	Altered Expression	[21]
SLCO1C1	TT340CE	Disputed	Biomarker	[22]
CXCL9	TTWE5PB	moderate	Biomarker	[23]
IL4	TTLGTKB	moderate	Biomarker	[24]
NFKB1	TTUIZKC	moderate	Genetic Variation	[25]
ABCC2	TTFLHJV	Strong	Biomarker	[22]
ABCC3	TTVLG21	Strong	Altered Expression	[26]
ABCG2	TTIMJ02	Strong	Biomarker	[27]
ALB	TTFNGC9	Strong	Biomarker	[28]
CCL5	TT9DWLC	Strong	Biomarker	[29]
CD58	TT5KSBY	Strong	Genetic Variation	[25]
CD80	TT89Z17	Strong	Biomarker	[30]
CD86	TT53XHB	Strong	Biomarker	[30]
CDH5	TTXLCFO	Strong	Biomarker	[23]
CDK12	TTJ21A9	Strong	Genetic Variation	[31]
CXCL10	TTQOVYA	Strong	Biomarker	[32]
CXCR5	TTIW59R	Strong	Biomarker	[33]
CYP21A2	TTP4GLG	Strong	Genetic Variation	[34]
EIF4E	TTZGCP6	Strong	Genetic Variation	[35]
FGF19	TTGCH11	Strong	Biomarker	[36]
GPBAR1	TTSDVTR	Strong	Biomarker	[37]
GRIK1	TT0MYE2	Strong	Genetic Variation	[35]
HIF1A	TTSN6QU	Strong	Biomarker	[38]
ICOSLG	TTB9Z8R	Strong	Biomarker	[33]
IGFBP1	TTCJTWF	Strong	Biomarker	[23]
IKZF3	TTCZVFZ	Strong	Genetic Variation	[39]
IL12B	TTGW72V	Strong	Biomarker	[33]
IL16	TTW4R0B	Strong	Biomarker	[25]
IL21R	TTZO9B0	Strong	Altered Expression	[25]
IL5	TTPREZD	Strong	Biomarker	[40]
IL7R	TTAWI51	Strong	Genetic Variation	[41]
KEAP1	TT3Z6Y9	Strong	Biomarker	[42]
LOX	TTQHNAM	Strong	Biomarker	[43]
LOXL2	TTFSUHX	Strong	Biomarker	[43]
LTBR	TTFO0PM	Strong	Genetic Variation	[44]
MAPK14	TTQBR95	Strong	Biomarker	[42]
MAPT	TTS87KH	Strong	Genetic Variation	[44]
NFE2L2	TTA6ZN2	Strong	Biomarker	[42]
NOS2	TTF10I9	Strong	Biomarker	[42]
NR1H4	TTS4UGC	Strong	Biomarker	[45]
OGDH	TTH8T6I	Strong	Biomarker	[46]
PLCB1	TTLPGU7	Strong	Genetic Variation	[35]
PRKCB	TTYPXQF	Strong	Genetic Variation	[41]
SH2B3	TT36N7Z	Strong	Genetic Variation	[44]
SLC10A1	TTWZRY5	Strong	Biomarker	[26]
SLC10A2	TTPI1M5	Strong	Biomarker	[47]
SLC22A1	TTM5Q4V	Strong	Genetic Variation	[48]
SLCO1B1	TTFGXEB	Strong	Altered Expression	[26]
STAT1	TTN7R6K	Strong	Genetic Variation	[49]
TNFRSF1A	TTG043C	Strong	Genetic Variation	[25]
TYK2	TTBYWP2	Strong	Genetic Variation	[50]
DPP8	TTJGLZF	Definitive	Altered Expression	[51]
DPP9	TTNDUL7	Definitive	Altered Expression	[51]
ITGA1	TTPERWV	Definitive	Altered Expression	[52]
KLRG1	TT299E6	Definitive	Biomarker	[53]
LRRC32	TT0FAYT	Definitive	Biomarker	[54]
PNPLA3	TTEUAEH	Definitive	Genetic Variation	[55]
POLR2G	TTD4YFP	Definitive	Genetic Variation	[56]
SLC25A1	TTTD730	Definitive	Biomarker	[57]
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⏷ Show the Full List of 78 DTT(s)

This Disease Is Related to 7 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
ABCC6	DT582KR	Limited	Biomarker	[9]
SLC17A8	DTAGDH7	Strong	Genetic Variation	[35]
SLC22A23	DTMJK12	Strong	Genetic Variation	[25]
SLC4A2	DTF7GAL	Strong	Altered Expression	[58]
SLC51A	DTMEQ32	Strong	Altered Expression	[59]
SLC51B	DT1V9AJ	Strong	Biomarker	[60]
SLC44A1	DT3I2SU	Definitive	Altered Expression	[48]
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⏷ Show the Full List of 7 DTP(s)

This Disease Is Related to 7 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
TPMT	DEFQ8VO	Disputed	Genetic Variation	[61]
UGT2B4	DENUPDX	Disputed	Altered Expression	[62]
CYP7A1	DEDZRQ1	Strong	Altered Expression	[63]
GPT	DER5HFI	Strong	Biomarker	[28]
MANBA	DEMH6UB	Strong	Genetic Variation	[50]
MMEL1	DEYCUQ2	Strong	Biomarker	[64]
SULT2A1	DE0P6LK	Strong	Biomarker	[65]
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⏷ Show the Full List of 7 DME(s)

This Disease Is Related to 120 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
DBT	OT4KZ5R9	Limited	Biomarker	[66]
DMBT1	OTVNU9D9	Limited	Altered Expression	[67]
HLA-DPA1	OT7OG7Y2	Limited	Genetic Variation	[68]
JPH3	OTHTJO2I	Limited	Biomarker	[69]
PDC	OT1UUVYY	Limited	Genetic Variation	[70]
PDP1	OT82RTMT	Limited	Biomarker	[71]
PRAM1	OTZN3DWD	Limited	Biomarker	[72]
PRPF40A	OT6EXJZN	Limited	Genetic Variation	[73]
RDX	OTNSYUN6	Limited	Biomarker	[9]
SLC12A9	OTR7VRAK	Limited	Biomarker	[74]
SS18L1	OT9ZEGV7	Limited	Biomarker	[75]
STAT4	OTAK3VFR	Limited	Biomarker	[52]
AQP4	OTA9MYD5	Disputed	Biomarker	[76]
CD180	OTITK5E6	Disputed	Altered Expression	[77]
COL1A1	OTI31178	Disputed	Therapeutic	[17]
CPEB1	OTLCXC6H	Disputed	Biomarker	[78]
DGCR2	OTEGL17Z	Disputed	Biomarker	[79]
GATM	OTIJ4Z11	Disputed	Genetic Variation	[80]
GNLY	OTZJKA8C	Disputed	Biomarker	[81]
HAP1	OT6SG0JQ	Disputed	Biomarker	[82]
HHIP	OT77RQYS	Disputed	Biomarker	[83]
NBPF3	OTRM16MN	Disputed	Altered Expression	[84]
NFYA	OTWFFOVH	Disputed	Biomarker	[82]
RBFOX1	OTFPKEL7	Disputed	Genetic Variation	[85]
SPINT1	OT1CLR5L	Disputed	Altered Expression	[86]
POU2AF1	OTOO6WHL	moderate	Genetic Variation	[41]
ACE	OTDF1964	Strong	Biomarker	[87]
ARHGAP31	OTUYTF4I	Strong	Genetic Variation	[31]
ARID3A	OTZZ4SFP	Strong	Genetic Variation	[25]
B3GAT1	OTXFP98E	Strong	Biomarker	[88]
BTG3	OT9ANHVT	Strong	Biomarker	[89]
C4A	OTXMOYXU	Strong	Biomarker	[90]
CCDC88B	OTEDUI2J	Strong	Genetic Variation	[50]
CD3D	OTRBLP0R	Strong	Biomarker	[23]
CD79A	OTOJC8DV	Strong	Biomarker	[91]
CLEC16A	OTLGV5SV	Strong	Genetic Variation	[50]
CNTN5	OTWU5FLZ	Strong	Genetic Variation	[35]
COL17A1	OTID5AH2	Strong	Genetic Variation	[35]
CTSZ	OTSCX2LL	Strong	Altered Expression	[92]
CYGB	OTX153DQ	Strong	Biomarker	[42]
DDX6	OTKWXVDY	Strong	Genetic Variation	[44]
DENND1B	OT3VCNPE	Strong	Genetic Variation	[50]
DGKQ	OT8OEPYT	Strong	Genetic Variation	[50]
DLST	OTBDF9HJ	Strong	Biomarker	[93]
EBAG9	OTTQLQCP	Strong	Biomarker	[94]
ELMO1	OTY2ORXK	Strong	Genetic Variation	[44]
ENTPD2	OTZ2PKPM	Strong	Biomarker	[95]
EXOC3L4	OTFD17IL	Strong	Genetic Variation	[50]
FBXL20	OTVTR1KY	Strong	Genetic Variation	[31]
GGTLC1	OTWJKUHQ	Strong	Altered Expression	[96]
GLI3	OTKDOE94	Strong	Genetic Variation	[35]
GSDMB	OTWA7P10	Strong	Genetic Variation	[97]
HLA-DOA	OTZE5Q7R	Strong	Genetic Variation	[98]
HLA-DRA	OT7KZMP2	Strong	Genetic Variation	[25]
HLA-DRB3	OT5PM9N7	Strong	Genetic Variation	[68]
ICAM3	OTTZ5A5D	Strong	Biomarker	[23]
IL12RB1	OTM1IJO2	Strong	Biomarker	[99]
IRF5	OT8SIIAP	Strong	Genetic Variation	[44]
KANSL1	OTYNSNNZ	Strong	Genetic Variation	[44]
KLHL1	OTAX6SAD	Strong	Biomarker	[26]
KRT7	OTLT3JFN	Strong	Biomarker	[100]
LGALS3BP	OT9AGQKH	Strong	Biomarker	[101]
LORICRIN	OTFRPVEO	Strong	Altered Expression	[43]
MED1	OTOO24C4	Strong	Genetic Variation	[31]
NAB1	OTEWHIZR	Strong	Genetic Variation	[50]
NELFCD	OTOLTZPU	Strong	Genetic Variation	[92]
NSA2	OTRU5QPO	Strong	Biomarker	[23]
NSF	OTKRJ2ZT	Strong	Genetic Variation	[44]
NUP210	OT3BLQ9M	Strong	Biomarker	[102]
ORMDL3	OTAB7MZF	Strong	Genetic Variation	[97]
PAX3	OTN5PJZV	Strong	Genetic Variation	[35]
PDZD2	OTPPVRGY	Strong	Biomarker	[40]
PLCL2	OTK5FDF6	Strong	Genetic Variation	[50]
POU2F1	OTK7ELJ0	Strong	Genetic Variation	[48]
PPARGC1A	OTHCDQ22	Strong	Altered Expression	[103]
PRDM16	OT0BGA27	Strong	Genetic Variation	[35]
PRICKLE1	OT9HHEM9	Strong	Genetic Variation	[31]
PSMD3	OT585WY8	Strong	Genetic Variation	[49]
PSMG4	OTWBC4YL	Strong	Genetic Variation	[25]
RAD51B	OTCJVRMY	Strong	Genetic Variation	[50]
RBM45	OTWTHD77	Strong	Genetic Variation	[104]
RELA	OTUJP9CN	Strong	Biomarker	[42]
RMI2	OTA0HP4S	Strong	Genetic Variation	[44]
RPL3	OTX6VXLB	Strong	Genetic Variation	[31]
RPS6KA4	OTTK8MUO	Strong	Genetic Variation	[31]
SDK1	OTX26906	Strong	Genetic Variation	[35]
SPIB	OTO4YKYI	Strong	Genetic Variation	[50]
SPPL2C	OTUTY2ED	Strong	Genetic Variation	[44]
STH	OTK8ULTH	Strong	Genetic Variation	[44]
SYCE1L	OTXU44F3	Strong	Biomarker	[26]
SYNGR1	OT0MBUK5	Strong	Genetic Variation	[105]
TIMMDC1	OTIK4I5R	Strong	Genetic Variation	[39]
TMEM39A	OTRT51D9	Strong	Genetic Variation	[25]
TNFSF8	OTDYGDJ3	Strong	Genetic Variation	[35]
TNPO3	OTOT3HH0	Strong	Genetic Variation	[44]
TSBP1	OT5GE8IO	Strong	Genetic Variation	[106]
AGBL2	OTCX9987	Definitive	Biomarker	[89]
BHMT	OTYB6PXZ	Definitive	Altered Expression	[48]
CCL24	OT9LGHV0	Definitive	Biomarker	[107]
CCL26	OT2B7HR9	Definitive	Altered Expression	[107]
CD1C	OT4XINUJ	Definitive	Biomarker	[108]
CD207	OTI9RUDN	Definitive	Biomarker	[108]
CDK2AP1	OTNFOHDJ	Definitive	Biomarker	[109]
CLDN14	OTS7GKOI	Definitive	Genetic Variation	[110]
CNGB1	OTA5DE38	Definitive	Biomarker	[54]
COG6	OTDLQITC	Definitive	Genetic Variation	[105]
COL9A3	OTCUJOEK	Definitive	Biomarker	[79]
FCRL3	OTIFXFWL	Definitive	Biomarker	[111]
INTU	OTXB13E6	Definitive	Biomarker	[112]
ITIH4	OT460OO1	Definitive	Biomarker	[113]
ITLN1	OT7ZLJVV	Definitive	Biomarker	[114]
JAM3	OTX0F9QL	Definitive	Altered Expression	[115]
PDK4	OTCMHMBZ	Definitive	Altered Expression	[103]
POGLUT1	OTDX7GZD	Definitive	Genetic Variation	[39]
RNASET2	OTWY64L7	Definitive	Genetic Variation	[116]
SCTR	OTC80IMR	Definitive	Genetic Variation	[117]
SIAE	OTDQUESH	Definitive	Genetic Variation	[118]
SP140L	OTL30JKT	Definitive	Biomarker	[119]
SPATA31A3	OTONL95V	Definitive	Biomarker	[54]
TNFAIP8L2	OTII0RM0	Definitive	Altered Expression	[120]
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⏷ Show the Full List of 120 DOT(s)

References

1	2016 FDA drug approvals. Nat Rev Drug Discov. 2017 Feb 2;16(2):73-76.
2	Primary biliary cholangitis: pathogenesis and therapeutic opportunities. Nat Rev Gastroenterol Hepatol. 2020 Feb;17(2):93-110.
3	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
4	ClinicalTrials.gov (NCT03394924) A Study to Assess the Safety, Tolerability, Pharmacokinetics and Efficacy of EDP-305 in Subjects With Primary Biliary Cholangitis. U.S. National Institutes of Health.
5	Association between serum soluble CD14 and IL-8 levels and clinical outcome in primary biliary cholangitis.Liver Int. 2017 Jun;37(6):897-905. doi: 10.1111/liv.13316. Epub 2016 Dec 6.
6	Phenotyping and auto-antibody production by liver-infiltrating B cells in primary sclerosing cholangitis and primary biliary cholangitis.J Autoimmun. 2017 Feb;77:45-54. doi: 10.1016/j.jaut.2016.10.003. Epub 2016 Oct 24.
7	Elevated expression of tyrosine kinase DDR2 in primary biliary cirrhosis.Autoimmunity. 2002 Dec;35(8):521-9. doi: 10.1080/0891693021000057784.
8	Identification of clinical risk factors for histological progression of primary biliary cholangitis.Hepatol Res. 2019 Sep;49(9):1015-1025. doi: 10.1111/hepr.13355. Epub 2019 Jun 14.
9	Changes in the expression and localization of hepatocellular transporters and radixin in primary biliary cirrhosis.J Hepatol. 2003 Nov;39(5):693-702. doi: 10.1016/s0168-8278(03)00410-0.
10	RANK/RANKL Acts as a Protective Factor by Targeting Cholangiocytes in Primary Biliary Cholangitis.Dig Dis Sci. 2020 Feb;65(2):470-479. doi: 10.1007/s10620-019-05758-5. Epub 2019 Aug 3.
11	Overexpression of Tumor Necrosis Factor-Like Ligand 1 A in Myeloid Cells Aggravates Liver Fibrosis in Mice.J Immunol Res. 2019 Feb 14;2019:7657294. doi: 10.1155/2019/7657294. eCollection 2019.
12	Upregulation of hepatic angiotensin-converting enzyme 2 (ACE2) and angiotensin-(1-7) levels in experimental biliary fibrosis.J Hepatol. 2007 Sep;47(3):387-95. doi: 10.1016/j.jhep.2007.03.008. Epub 2007 Apr 2.
13	Angiotensin-(1-7), an alternative metabolite of the renin-angiotensin system, is up-regulated in human liver disease and has antifibrotic activity in the bile-duct-ligated rat.Clin Sci (Lond). 2009 Sep 14;117(11):375-86. doi: 10.1042/CS20080647.
14	Dystroglycan expression in hepatic stellate cells: role in liver fibrosis.Lab Invest. 2002 Aug;82(8):1053-61. doi: 10.1097/01.lab.0000024429.73158.de.
15	Angiotensin-II type 1 receptor-mediated Janus kinase 2 activation induces liver fibrosis.Hepatology. 2014 Jul;60(1):334-48. doi: 10.1002/hep.27117. Epub 2014 May 6.
16	Structure of thymic cysts in congenital lymph nodes-lacking mice.Anat Histol Embryol. 2008 Apr;37(2):126-30. doi: 10.1111/j.1439-0264.2007.00806.x. Epub 2007 Nov 6.
17	Macrophage-mediated phagocytosis of apoptotic cholangiocytes contributes to reversal of experimental biliary fibrosis.Am J Physiol Gastrointest Liver Physiol. 2010 Mar;298(3):G323-34. doi: 10.1152/ajpgi.00394.2009. Epub 2010 Jan 7.
18	Effect of chronic administration of sildenafil on sodium retention and on the hemodynamic complications associated with liver cirrhosis in the rat.Eur J Pharmacol. 2007 Oct 15;572(1):49-56. doi: 10.1016/j.ejphar.2007.05.068. Epub 2007 Jun 13.
19	Prevention of acute kidney injury in a rodent model of cirrhosis following selective gut decontamination is associated with reduced renal TLR4 expression.J Hepatol. 2012 May;56(5):1047-1053. doi: 10.1016/j.jhep.2011.11.024. Epub 2012 Jan 17.
20	Long-term treatment of bile duct-ligated rats with rapamycin (sirolimus) significantly attenuates liver fibrosis: analysis of the underlying mechanisms.J Pharmacol Exp Ther. 2005 Jun;313(3):952-61. doi: 10.1124/jpet.104.079616. Epub 2005 Mar 15.
21	Tumor transcriptome reveals the predictive and prognostic impact of lysosomal protease inhibitors in non-small-cell lung cancer.J Clin Oncol. 2006 Apr 10;24(11):1729-44. doi: 10.1200/JCO.2005.03.3399. Epub 2006 Mar 20.
22	Magnetic resonance imaging with hepatospecific contrast agents in cirrhotic rat livers.Invest Radiol. 2005 Apr;40(4):187-94. doi: 10.1097/01.rli.0000154587.00638.77.
23	Gene expression profiling of early primary biliary cirrhosis: possible insights into the mechanism of action of ursodeoxycholic acid. Liver Int. 2008 Aug;28(7):997-1010. doi: 10.1111/j.1478-3231.2008.01744.x. Epub 2008 Apr 15.
24	Implication of increased serum stromal cell-derived factor-1 for primary biliary cholangitis.Int Immunopharmacol. 2018 Mar;56:285-290. doi: 10.1016/j.intimp.2018.01.046. Epub 2018 Feb 3.
25	A genome-wide association study identifies six novel risk loci for primary biliary cholangitis.Nat Commun. 2017 Apr 20;8:14828. doi: 10.1038/ncomms14828.
26	Adaptive changes in hepatobiliary transporter expression in primary biliary cirrhosis.J Hepatol. 2003 Jun;38(6):717-27. doi: 10.1016/s0168-8278(03)00096-5.
27	Influence of biliary cirrhosis on the detoxification and elimination of a food derived carcinogen.Gut. 2004 Dec;53(12):1850-5. doi: 10.1136/gut.2003.037507.
28	Concomitant systemic lupus erythematosus might have a negative impact on the biochemical responses to treatment in patients with primary biliary cholangitis.Clin Rheumatol. 2020 Mar;39(3):795-803. doi: 10.1007/s10067-019-04853-2. Epub 2019 Dec 6.
29	Fibrates suppress chenodeoxycholic acid-induced RANTES expression through inhibition of NF-kappaB activation. Eur J Pharmacol. 2002 Jul 12;448(1):19-26. doi: 10.1016/s0014-2999(02)01902-7.
30	Therapeutic trials of biologics in primary biliary cholangitis: An open label study of abatacept and review of the literature.J Autoimmun. 2019 Jul;101:26-34. doi: 10.1016/j.jaut.2019.04.005. Epub 2019 Apr 24.
31	Genome-wide association study identifies 12 new susceptibility loci for primary biliary cirrhosis.Nat Genet. 2011 Mar 13;43(4):329-32. doi: 10.1038/ng.789.
32	CXCR3 axis in patients with primary biliary cirrhosis: a possible novel mechanism of the effect of ursodeoxycholic acid.Clin Exp Immunol. 2013 Apr;172(1):9-15. doi: 10.1111/cei.12032.
33	Disease susceptibility genes shared by primary biliary cirrhosis and Crohn's disease in the Japanese population.J Hum Genet. 2015 Sep;60(9):525-31. doi: 10.1038/jhg.2015.59. Epub 2015 Jun 18.
34	Genome-wide Association Studies of Specific Antinuclear Autoantibody Subphenotypes in Primary Biliary Cholangitis.Hepatology. 2019 Jul;70(1):294-307. doi: 10.1002/hep.30604. Epub 2019 Apr 29.
35	Genome-wide association study identifies TNFSF15 and POU2AF1 as susceptibility loci for primary biliary cirrhosis in the Japanese population.Am J Hum Genet. 2012 Oct 5;91(4):721-8. doi: 10.1016/j.ajhg.2012.08.010. Epub 2012 Sep 20.
36	Comprehensive Analysis of Serum and Fecal Bile Acid Profiles and Interaction with Gut Microbiota in Primary Biliary Cholangitis.Clin Rev Allergy Immunol. 2020 Feb;58(1):25-38. doi: 10.1007/s12016-019-08731-2.
37	Bile acid receptors in the biliary tree: TGR5 in physiology and disease.Biochim Biophys Acta Mol Basis Dis. 2018 Apr;1864(4 Pt B):1319-1325. doi: 10.1016/j.bbadis.2017.08.021. Epub 2017 Aug 25.
38	Hypoxia-inducible factor activation in myeloid cells contributes to the development of liver fibrosis in cholestatic mice.J Pharmacol Exp Ther. 2012 May;341(2):307-16. doi: 10.1124/jpet.111.189340. Epub 2012 Jan 23.
39	POGLUT1, the putative effector gene driven by rs2293370 in primary biliary cholangitis susceptibility locus chromosome 3q13.33.Sci Rep. 2019 Jan 14;9(1):102. doi: 10.1038/s41598-018-36490-1.
40	Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis.Hepatology. 2007 Jun;45(6):1538-46. doi: 10.1002/hep.21697.
41	Genome-wide association studies identify PRKCB as a novel genetic susceptibility locus for primary biliary cholangitis in the Japanese population.Hum Mol Genet. 2017 Feb 1;26(3):650-659. doi: 10.1093/hmg/ddw406.
42	Hepatoprotective effects of diosmin and/or sildenafil against cholestatic liver cirrhosis: The role of Keap-1/Nrf-2 and P(38)-MAPK/NF-B/iNOS signaling pathway.Food Chem Toxicol. 2018 Oct;120:294-304. doi: 10.1016/j.fct.2018.07.027. Epub 2018 Jul 17.
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