MCID: PRM006
MIFTS: 54

Primary Biliary Cirrhosis

Categories: Endocrine diseases, Gastrointestinal diseases, Liver diseases

Aliases & Classifications for Primary Biliary Cirrhosis

MalaCards integrated aliases for Primary Biliary Cirrhosis:

Name: Primary Biliary Cirrhosis 12 75 37 15 38 17 72 33
Biliary Liver Cirrhosis 12 17
Chronic Nonsuppurative Destructive Cholangitis 12
Biliary Cirrhosis Primary 55
Liver Cirrhosis, Biliary 44
Cholestatic Cirrhosis 12
Biliary Cirrhosis 72

Classifications:



External Ids:

Disease Ontology 12 DOID:12236
KEGG 37 H01467
ICD9CM 35 571.6
MeSH 44 D008105
SNOMED-CT 68 1761006 31712002
ICD10 33 K74.3 K74.5
UMLS 72 C0008312 C0023892

Summaries for Primary Biliary Cirrhosis

KEGG : 37
Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, eventually leading to fibrosis and potential cirrhosis. Patients are usually middle-aged women and presents in the fifth or sixth decade of life. The most common symptoms are pruritus. It is not clear what causes PBC. It may be relate to problems in the autoimmune mechanism. The increased risk of genetic predisposition and environmental factors (infectious or toxic agents) trigger the disease. Ursodeoxycholic acid is currently the only medicine approved for the treatment of the disease.

MalaCards based summary : Primary Biliary Cirrhosis, also known as biliary liver cirrhosis, is related to systemic lupus erythematosus and multiple sclerosis. An important gene associated with Primary Biliary Cirrhosis is DLAT (Dihydrolipoamide S-Acetyltransferase), and among its related pathways/superpathways are MicroRNAs in cancer and Parkinsons Disease Pathway. The drugs Abatacept and chenodeoxycholic acid have been mentioned in the context of this disorder. Affiliated tissues include Umbilical Cord, liver and t cells.

Disease Ontology : 12 A liver cirrhosis characterized by chronic and slow progressive destruction of intrahepatic bile ducts.

Wikipedia : 75 Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune... more...

Related Diseases for Primary Biliary Cirrhosis

Diseases in the Primary Biliary Cirrhosis family:

Biliary Cirrhosis, Primary, 1 Biliary Cirrhosis, Primary, 2
Biliary Cirrhosis, Primary, 3 Biliary Cirrhosis, Primary, 4
Biliary Cirrhosis, Primary, 5

Diseases related to Primary Biliary Cirrhosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 572)
# Related Disease Score Top Affiliating Genes
1 systemic lupus erythematosus 30.5 MIR346 MIR30D MIR296 MIR223 MIR197 MIR150
2 multiple sclerosis 30.4 MIR326 MIR223 MIR20A MIR17
3 psoriasis 30.1 MIR326 MIR20A MIR197 MIR133B MIR122
4 muscular dystrophy, duchenne type 29.7 MIR361 MIR30D
5 glioblastoma 29.6 MIR451A MIR326 MIR296 MIR197
6 gastric cancer 29.2 MIR451A MIR223 MIR20A MIR150 MIR122
7 primary biliary cholangitis 12.8
8 reynolds syndrome 12.7
9 biliary cirrhosis, primary, 1 12.2
10 biliary cirrhosis, primary, 4 11.8
11 biliary cirrhosis, primary, 5 11.8
12 biliary cirrhosis, primary, 2 11.8
13 biliary cirrhosis, primary, 3 11.8
14 liver cirrhosis 11.7
15 cholangitis 11.2
16 liver disease 11.2
17 autoimmune disease 11.1
18 cholestasis 11.1
19 autoimmune hepatitis 11.1
20 sclerosing cholangitis 11.0
21 cholangitis, primary sclerosing 11.0
22 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.9
23 varicose veins 10.9
24 portal hypertension 10.9
25 systemic scleroderma 10.8
26 viral hepatitis 10.8
27 scleroderma, familial progressive 10.8
28 esophageal varix 10.7
29 raynaud phenomenon 10.7
30 hepatocellular carcinoma 10.7
31 osteoporosis 10.7
32 thyroiditis 10.7
33 crest syndrome 10.7
34 sjogren syndrome 10.7
35 bone disease 10.7
36 sarcoidosis 1 10.7
37 bone mineral density quantitative trait locus 8 10.7
38 bone mineral density quantitative trait locus 15 10.7
39 polymyositis 10.6
40 hypercholesterolemia, familial, 1 10.6
41 osteomalacia 10.6
42 hepatitis 10.6
43 deficiency anemia 10.6
44 connective tissue disease 10.6
45 hemolytic anemia 10.5
46 graft-versus-host disease 10.5
47 renal tubular acidosis 10.5
48 ulcerative colitis 10.5
49 splenomegaly 10.5
50 telangiectasis 10.5

Comorbidity relations with Primary Biliary Cirrhosis via Phenotypic Disease Network (PDN):


Active Peptic Ulcer Disease Acute Cystitis
Cholangitis Deficiency Anemia
Hepatic Encephalopathy Hypothyroidism
Portal Hypertension

Graphical network of the top 20 diseases related to Primary Biliary Cirrhosis:



Diseases related to Primary Biliary Cirrhosis

Symptoms & Phenotypes for Primary Biliary Cirrhosis

Drugs & Therapeutics for Primary Biliary Cirrhosis

Drugs for Primary Biliary Cirrhosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 162)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Abatacept Approved Phase 4 332348-12-6 10237
2
chenodeoxycholic acid Approved Phase 4 474-25-9 10133
3
Sorafenib Approved, Investigational Phase 4 284461-73-0 216239 406563
4 Cathartics Phase 4
5 Laxatives Phase 4
6 Protein Kinase Inhibitors Phase 4
7
Fenofibrate Approved Phase 3 49562-28-9 3339
8
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
9
leucovorin Approved Phase 3 58-05-9 143 6006
10
Colchicine Approved Phase 3 64-86-8 6167 2833
11
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
12
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
13
Molybdenum Approved Phase 3 7439-98-7 185498
14
Copper Approved, Investigational Phase 3 7440-50-8 27099
15
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
16
Atorvastatin Approved Phase 3 134523-00-5 60823
17
Metformin Approved Phase 2, Phase 3 657-24-9 4091 14219
18
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
19
Budesonide Approved Phase 3 51333-22-3 63006 5281004
20
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
21
Taurochenodeoxycholic acid Experimental Phase 3 516-35-8 387316
22
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
23
Tetrathiomolybdate Investigational Phase 3 16330-92-0
24 Folic Acid Antagonists Phase 3
25 Folate Phase 3
26 Vitamin B Complex Phase 3
27 Vitamin B9 Phase 3
28 Dermatologic Agents Phase 3
29 Antimetabolites, Antineoplastic Phase 3
30 Tubulin Modulators Phase 3
31 Antimitotic Agents Phase 3
32 Lamivudine, zidovudine drug combination Phase 2, Phase 3
33 Angiogenesis Inhibitors Phase 3
34 Angiogenesis Modulating Agents Phase 3
35 Chelating Agents Phase 3
36 Lipid Regulating Agents Phase 3
37 Hypolipidemic Agents Phase 3
38 Antimetabolites Phase 3
39 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
40 Anticholesteremic Agents Phase 3
41 carnitine Phase 3
42 Hypoglycemic Agents Phase 2, Phase 3
43 Hormone Antagonists Phase 3
44 Hormones Phase 3
45 Anti-Inflammatory Agents Phase 3
46 glucocorticoids Phase 3
47 Respiratory System Agents Phase 3
48 Peripheral Nervous System Agents Phase 3
49 Anti-Asthmatic Agents Phase 3
50 Autonomic Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 140)
# Name Status NCT ID Phase Drugs
1 Double-blind, Double-dummy, Randomised, Crossover, Multicentre Phase IV Clinical Study Comparing the Effect of Ursofalk 500 mg Tablets od Versus Ursofalk 250 mg Capsules od on Liver Enzymes in the Treatment of Primary Biliary Cirrhosis Completed NCT01510860 Phase 4 UDCA (Ursodeoxycholic acid);UDCA (Ursodeoxycholic acid)
2 Abatacept For The Treatment Of Primary Biliary Cirrhosis With An Incomplete Biochemical Response To Ursodeoxycholic Acid Completed NCT02078882 Phase 4
3 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
4 A Phase 4, Double-Blind, Randomized, Placebo-Controlled Study Evaluating the Pharmacokinetics and Safety of Obeticholic Acid in Patients With Primary Biliary Cholangitis and Moderate to Severe Hepatic Impairment Recruiting NCT03633227 Phase 4 Obeticholic Acid (OCA);Placebo
5 A Phase 4, Double-Blind, Randomized, Placebo-Controlled, Multicenter Study Evaluating the Effect of Obeticholic Acid on Clinical Outcomes in Patients With Primary Biliary Cholangitis Recruiting NCT02308111 Phase 4 Obeticholic Acid (OCA);Placebo
6 A Randomized Controlled Trial of 18-22mg/kg/d Ursodeoxycholic in Refractory Primary Biliary Cholangitis Recruiting NCT03345589 Phase 4 18-22mg/kg/d Ursodeoxycholic;13-15mg/kg/d Ursodeoxycholic
7 Mechanism of Sorafenib Resistance in Patients With Advanced Hepatocellular Carcinoma Recruiting NCT02733809 Phase 4 Sorafenib
8 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis: a Randomized Control Study Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
9 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
10 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02916290 Phase 3 Fuzhenghuayu;UDCA
11 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
12 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
13 Effectiveness of Ursodeoxycholic Acid Use in the Prevention of Gallstone Formation After Sleeve Gastrectomy: A Prospective Randomized Multicenter Placebo-Controlled Trial Unknown status NCT02721862 Phase 3 Ursodeoxycholic Acid;Placebo
14 Multicenter Randomized Trial Comparing Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis Unknown status NCT01398917 Phase 3
15 Probiotics in Patients With Primary Sclerosing Cholangitis and Inflammatory Bowel Disease- a Randomized Placebo-Controlled Cross-Over Trial Unknown status NCT00161148 Phase 3 Probiotics
16 A Phase 3, Double Blind, Placebo Controlled Trial and Long Term Safety Extension of Obeticholic Acid in Patients With Primary Biliary Cirrhosis Completed NCT01473524 Phase 3 Obeticholic Acid (OCA);Placebo
17 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
18 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Low-Dose Oral Methotrexate Versus Colchicine for Primary Biliary Cirrhosis Completed NCT00004748 Phase 3 colchicine;methotrexate;ursodiol
19 A Randomized, Double-blinded, Double-dummy, Parallel-controlled and Multicenter Clinical Trial to Investigate Safety and Efficacy of Tauroursodeoxycholic Acid Capsules in Treatment of Adult Primary Biliary Cirrhosis Completed NCT01857284 Phase 3 Tauroursodeoxycholic Acid Capsules;Ursodeoxycholic Acid Capsules
20 Randomized Controlled Pilot Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
21 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
22 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
23 Phase III Trial of Tetrathiomolybdate (TM) in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
24 Multicenter, Randomized, Double-blind Placebo Controlled Trial of Bezafibrate for the Treatment of Primary Biliary Cirrhosis in Patients With Incomplete Response to Ursodesoxycholic Acid Therapy. Completed NCT01654731 Phase 3 Bezafibrate;placebo
25 Evaluate the Efficacy And Safety Of TUDCA Compare UDCA In The Treatment Of Cholestatic Liver Disease-PBC by A Randomized,Double-Blind,Double Dummy,Parallel-Controlled,Multicenter Trial and The Consecutive Treatment By TUDCA Completed NCT01829698 Phase 3 tauroursodeoxycholic;ursodeoxycholic acid
26 A Phase 3 Study to Evaluate the Place of Budesonide in the Treatment of GVHD Completed NCT00128739 Phase 3 Budesonide
27 Efficacy and Security of Bezafibrate in Patients With Primary Biliary Cirrhosis Without Biochemical Response to Ursodeoxycholic Acid: A Randomized, Double-blind, Placebo-controlled Trial Recruiting NCT02937012 Phase 3 Bezafibrate;Ursodeoxycholic Acid;Placebo (for Bezafibrate)
28 An Open Label Long-Term Study to Evaluate the Safety and Tolerability of Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) Recruiting NCT03301506 Phase 2, Phase 3 Seladelpar 2 mg Capsule;Seladelpar 5 mg Capsule;Seladelpar 10 mg Capsule
29 A 52-week, Placebo-controlled, Randomized, Phase 3 Study to Evaluate the Safety and Efficacy of Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) and an Inadequate Response to or an Intolerance to Ursodeoxycholic Acid (UDCA) Recruiting NCT03602560 Phase 3 seladelpar 5-10 mg;seladelpar 10 mg;Placebo
30 Estudio clínico Fase III Para Evaluar la Eficacia terapéutica en Pacientes Mexicanos Con Dislipidemia Mediante el Uso vía Oral de L-Carnitina + Atorvastatina Comparado Con Atorvastatina Active, not recruiting NCT03696940 Phase 3 L-Carnitine 500Mg Oral Tablet + Atorvastatin 10 mg;Atorvastatin 10mg
31 METformin in Intrahepatic Cholestasis of Pregnancy (METRIC) Study Not yet recruiting NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
32 Double-blind, Randomised, Placebo-controlled, Multi-centre Phase III Clinical Study Comparing the Combination of Ursodeoxycholic Acid Capsules Plus Budesonide Capsules to Ursodeoxycholic Acid Capsules Plus Placebo in the Treatment of Primary Biliary Cirrhosis Terminated NCT00746486 Phase 3 budesonide;budesonide placebo
33 A Phase I/II, Open Label, Multicenter, Pilot Dose Escalation Study to Evaluate the Safety, Tolerability and Pharmacodynamics of FFP104 in Subjects Previously Diagnosed With Primary Biliary Cirrhosis (PBC) Unknown status NCT02193360 Phase 1, Phase 2 FFP104
34 Phase 1/2 Study of UC-MSC Treatment for Evaluation the Efficacy and Safety in Patients With Primary Biliary Cirrhosis Unknown status NCT01662973 Phase 1, Phase 2
35 A Randomized Controlled Clinical Trial on the Efficacy and Safety of Fenofibrate Combined With Ursodeoxycholic Acid in PBC Patients With an Incomplete Biochemical Response to UDCA Unknown status NCT02965911 Phase 1, Phase 2 Fenofibrate;UDCA
36 Open-Labelled, Multicenter Phase II Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
37 A Phase 2, Randomized, Double Blind, Placebo Controlled, Parallel Group, Multiple Center Study to Evaluate the Safety, Tolerability, and Pharmacodynamic Activity of NGM282 in Combination With Ursodeoxycholic Acid (UDCA) Administered for 28 Days in Patients With Primary Biliary Cirrhosis Completed NCT02026401 Phase 2
38 A Phase 2, Multi-Center Study to Evaluate Three Doses of NGM282 Administered for 24 Weeks in Patients With Primary Biliary Cirrhosis (PBC) Who Have Completed Study 13-0103 Completed NCT02135536 Phase 2
39 A Study of INT 747 (6α-ethyl Chenodeoxycholic Acid (6-ECDCA)) in Combination With Ursodeoxycholic Acid (URSO®, UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT00550862 Phase 2 INT-747;Ursodeoxycholic Acid (URSO);Placebo
40 A Study of INT-747 (6-ECDCA) Monotherapy in Patients With Primary Biliary Cirrhosis Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
41 A Phase 2, Randomized, Double-blind, Placebo-controlled Study to Evaluate LUM001, an Apical Sodium-dependent Bile Acid Transporter Inhibitor (ASBTi) in Combination With Ursodeoxycholic Acid (UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT01904058 Phase 2 LUM001;Placebo;Ursodeoxycholic Acid
42 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
43 Pilot Study of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
44 A Pilot Study of Pentoxifylline for the Treatment of Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
45 Open-Label Pilot Investigation of Moexipril for the Treatment of Primary Biliary Cirrhosis (PBC) Completed NCT00588302 Phase 2 Moexipril
46 A Multi-part, Randomized, Double-blind, Placebo-controlled Study to Assess the Safety, Tolerability and Efficacy of Tropifexor (LJN452) in Patients With Primary Biliary Cholangitis Completed NCT02516605 Phase 2 Part 1: LJN452;Part 1: Placebo;Part 2: LJN452 Dose level 1;Part 2: Placebo;Part 2: LJN452 Dose level 2
47 A Phase 2 Clinical Trial Investigating the Effects of Obeticholic Acid on Lipoprotein Metabolism in Subjects With Primary Biliary Cirrhosis Completed NCT01865812 Phase 2 obeticholic acid (OCA)
48 Effects of Rituximab (Rituxan) on B Cell and AMA Response in Patients With Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
49 A Phase 2, Multi-center, Randomized, Double-blind, Placebo-controlled, Parallel-group Study Evaluating the Efficacy and Safety of Ustekinumab in Subjects With Primary Biliary Cirrhosis Who Had an Inadequate Response to Ursodeoxycholic Acid (UDCA) Completed NCT01389973 Phase 2 ustekinumab 90 mg;ustekinumab 45 mg;ustekinumab 180 mg;Placebo
50 A Randomised, Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Repeat Doses of GSK2330672 Administration in Patients With Primary Biliary Cirrhosis (PBC) and Symptoms of Pruritus Completed NCT01899703 Phase 2 GSK2330672;Placebo;Ursodeoxycholic acid

Search NIH Clinical Center for Primary Biliary Cirrhosis

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Colchicine
Penicillamine
Ursodiol

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Primary Biliary Cirrhosis cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: liver cirrhosis, biliary

Genetic Tests for Primary Biliary Cirrhosis

Anatomical Context for Primary Biliary Cirrhosis

MalaCards organs/tissues related to Primary Biliary Cirrhosis:

41
Liver, T Cells, Bone, Testes, B Cells, Thyroid, Endothelial
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Primary Biliary Cirrhosis:
# Tissue Anatomical CompartmentCell Relevance
1 Umbilical Cord Wharton's Jelly Mesenchymal Stem Cells Potential therapeutic candidate

Publications for Primary Biliary Cirrhosis

Articles related to Primary Biliary Cirrhosis:

(show top 50) (show all 7555)
# Title Authors PMID Year
1
Primary biliary cirrhosis is associated with altered hepatic microRNA expression. 38 88
19345069 2009
2
WITHDRAWN:MicroRNA profile in peripheral blood T cells of patients with primary biliary cirrhosis. 38 88
18631158 2008
3
LBR mutation and nuclear envelope defects in a patient affected with Reynolds syndrome. 9 38
20522425 2010
4
Catalytic domain of PDC-E2 contains epitopes recognized by antimitochondrial antibodies in primary biliary cirrhosis. 9 38
20180236 2010
5
Diagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease. 9 38
20101770 2010
6
Mycoplasma antigens as a possible trigger for the induction of antimitochondrial antibodies in primary biliary cirrhosis. 9 38
19638108 2009
7
Vitamin D receptor polymorphisms in primary biliary cirrhosis: a functional connection? 9 38
19398232 2009
8
The pathogenesis of primary biliary cirrhosis. 9 38
19630465 2009
9
Vitamin D receptor polymorphisms are associated with increased susceptibility to primary biliary cirrhosis in Japanese and Italian populations. 9 38
19376604 2009
10
Farnesoid X receptor agonists in biliary tract disease. 9 38
19300246 2009
11
Bile salts control the antimicrobial peptide cathelicidin through nuclear receptors in the human biliary epithelium. 9 38
19245866 2009
12
CTLA4/ICOS gene variants and haplotypes are associated with rheumatoid arthritis and primary biliary cirrhosis in the Canadian population. 9 38
19333938 2009
13
When lightning strikes twice: the plot thickens for a dual role of the anion exchanger 2 (AE2/SLC4A2) in the pathogenesis and treatment of primary biliary cirrhosis. 9 38
19157624 2009
14
Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization. 9 38
19094117 2009
15
Apotopes and the biliary specificity of primary biliary cirrhosis. 9 38
19185000 2009
16
Decreased production of immunoglobulin M and A in autoimmune pancreatitis. 9 38
19626266 2009
17
Xenobiotic incorporation into pyruvate dehydrogenase complex can occur via the exogenous lipoylation pathway. 9 38
19003917 2008
18
Primary biliary cirrhosis is associated with a genetic variant in the 3' flanking region of the CTLA4 gene. 9 38
18778710 2008
19
Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis. 9 38
18644655 2008
20
Isoform of fibronectin mediates bone loss in patients with primary biliary cirrhosis by suppressing bone formation. 9 38
18348696 2008
21
Increased expression of Toll-like receptor 3 in intrahepatic biliary epithelial cells at sites of ductular reaction in diseased livers. 9 38
19669308 2008
22
Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. 9 38
18471521 2008
23
Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis. 9 38
18041714 2008
24
Breakthrough of immune self-tolerance to calreticulin induced by CpG-oligodeoxynucleotides as adjuvant. 9 38
18402289 2007
25
Clinical and serological features of patients with autoantibodies to GW/P bodies. 9 38
17870671 2007
26
Association of CTLA4 single nucleotide polymorphisms with viral but not autoimmune liver disease. 9 38
18049163 2007
27
Nuclear envelope protein autoantigens in primary biliary cirrhosis. 9 38
17931195 2007
28
Intrahepatic expression of the co-stimulatory molecules programmed death-1, and its ligands in autoimmune liver disease. 9 38
17610472 2007
29
High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. 9 38
17498256 2007
30
Increased serum anti-carbonic anhydrase II antibodies in patients with Graves' disease. 9 38
17516290 2007
31
Differential epitope mapping of antibodies to PDC-E2 in patients with hematologic malignancies after allogeneic hematopoietic stem cell transplantation and primary biliary cirrhosis. 9 38
17068145 2007
32
Pathogenesis of autoimmune hepatitis: from break of tolerance to immune-mediated hepatocyte apoptosis. 9 38
17320796 2007
33
AMA production in primary biliary cirrhosis is promoted by the TLR9 ligand CpG and suppressed by potassium channel blockers. 9 38
17256753 2007
34
MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. 9 38
17295178 2007
35
Primary biliary cirrhosis in female subjects with sicca-associated antibodies. 9 38
18084701 2007
36
Possible association of cytotoxic T lymphocyte antigen-4 genetic polymorphism with liver damage of primary biliary cirrhosis in Japan. 9 38
17427759 2006
37
Cytokeratin-7, cytokeratin-19, and c-Kit: Immunoreaction during the evolution stages of primary biliary cirrhosis. 9 38
16963314 2006
38
IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis. 9 38
17058261 2006
39
Transgenic mice aberrantly expressing pyruvate dehydrogenase complex E2 component on biliary epithelial cells do not show primary biliary cirrhosis. 9 38
16792678 2006
40
PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis. 9 38
16629643 2006
41
Altered expression of alkaline phosphatase (ALP) in the liver of primary biliary cirrhosis (PBC) patients. 9 38
16567122 2006
42
Anti-p97/VCP antibodies: an autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease? 9 38
16640662 2006
43
A novel multidrug-resistance protein 2 gene mutation identifies a subgroup of patients with primary biliary cirrhosis and pruritus. 9 38
16628674 2006
44
Cholestasis and cholestatic syndromes. 9 38
16550034 2006
45
Multidrug resistance 1 genotype and disposition of budesonide in early primary biliary cirrhosis. 9 38
16584389 2006
46
Expression of epithelial cellular adhesion molecule (Ep-CAM) in chronic (necro-)inflammatory liver diseases and hepatocellular carcinoma. 9 38
16364680 2006
47
Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis. 9 38
16129198 2005
48
Primary biliary cirrhosis is characterized by IgG3 antibodies cross-reactive with the major mitochondrial autoepitope and its Lactobacillus mimic. 9 38
16025495 2005
49
Enhanced expression of type I interferon and toll-like receptor-3 in primary biliary cirrhosis. 9 38
15856047 2005
50
Patients with primary biliary cirrhosis have increased serum total antioxidant capacity measured with the crocin bleaching assay. 9 38
16015688 2005

Variations for Primary Biliary Cirrhosis

Expression for Primary Biliary Cirrhosis

Search GEO for disease gene expression data for Primary Biliary Cirrhosis.

Pathways for Primary Biliary Cirrhosis

Pathways related to Primary Biliary Cirrhosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.85 MIR483 MIR451A MIR326 MIR324 MIR30D MIR223
2 11.06 MIR212 MIR20A MIR17
3 10.74 MIR150 MIR133B

GO Terms for Primary Biliary Cirrhosis

Cellular components related to Primary Biliary Cirrhosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.36 MIR451A MIR361 MIR328 MIR30D MIR223 MIR212

Biological processes related to Primary Biliary Cirrhosis according to GeneCards Suite gene sharing:

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 gene silencing by miRNA GO:0035195 9.55 MIR483 MIR451A MIR361 MIR346 MIR328 MIR326
2 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.51 MIR20A MIR17
3 negative regulation of blood vessel endothelial cell migration GO:0043537 9.49 MIR212 MIR133B
4 positive regulation of phagocytosis GO:0050766 9.48 MIR20A MIR17
5 positive regulation of cell migration involved in sprouting angiogenesis GO:0090050 9.46 MIR296 MIR150
6 negative regulation of sprouting angiogenesis GO:1903671 9.43 MIR20A MIR17
7 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.43 MIR483 MIR361 MIR20A
8 miRNA mediated inhibition of translation GO:0035278 9.43 MIR483 MIR299 MIR212 MIR20A MIR17 MIR133B
9 negative regulation of blood vessel endothelial cell proliferation involved in sprouting angiogenesis GO:1903588 9.4 MIR483 MIR361
10 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.37 MIR451A MIR20A
11 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.32 MIR20A MIR17
12 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.16 MIR20A MIR17

Molecular functions related to Primary Biliary Cirrhosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.36 MIR483 MIR361 MIR328 MIR299 MIR296 MIR223

Sources for Primary Biliary Cirrhosis

3 CDC
7 CNVD
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10 dbSNP
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28 GO
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30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
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44 MeSH
45 MESH via Orphanet
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49 NCI
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57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
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73 UMLS via Orphanet
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