Immunologic Responses and the Pathophysiology of Primary Biliary Cholangitis.
Apoptosis
Bile acids
Biliary epithelial cell
Immune tolerance
Microbiome
Senescence
X chromosome
Journal
Clinics in liver disease
ISSN: 1557-8224
Titre abrégé: Clin Liver Dis
Pays: United States
ID NLM: 9710002
Informations de publication
Date de publication:
11 2022
11 2022
Historique:
entrez:
21
10
2022
pubmed:
22
10
2022
medline:
26
10
2022
Statut:
ppublish
Résumé
Primary biliary cholangitis (PBC) is an autoimmune liver disease with a female predisposition and selective destruction of intrahepatic small bile ducts leading to nonsuppurative destructive cholangitis. It is characterized by seropositivity of antimitochondrial antibodies or PBC-specific antinuclear antibodies, progressive cholestasis, and typical liver histologic manifestations. Destruction of the protective bicarbonate-rich umbrella is attributed to the decreased expression of membrane transporters in biliary epithelial cells (BECs), leading to the accumulation of hydrophobic bile acids and sensitizing BECs to apoptosis. A recent X-wide association study reveals a novel risk locus on the X chromosome, which reiterates the importance of Treg cells.
Identifiants
pubmed: 36270718
pii: S1089-3261(22)00041-1
doi: 10.1016/j.cld.2022.06.003
pii:
doi:
Substances chimiques
Bicarbonates
0
Antibodies, Antinuclear
0
Bile Acids and Salts
0
Membrane Transport Proteins
0
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
583-611Informations de copyright
Copyright © 2022 Elsevier Inc. All rights reserved.