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Article

Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells.

American journal of physiology. Gastrointestinal and liver physiology - 1 Jan 2008

Kagawa Tatehiro, Watanabe Norihito, Mochizuki Kaori, Numari Asano, Ikeno Yoshie, Itoh Johbu, Tanaka Hirotoshi, Arias Irwin M, Mine Tetsuya

Abstract excerpt

Progressive familial cholestasis (PFIC) 2 and benign recurrent intrahepatic cholestasis (BRIC) 2 are caused by mutations in the bile salt export pump (BSEP, ABCB11) gene; however, their prognosis differs. PFIC2 progresses to cirrhosis and requires liver transplantation, whereas BRIC2 is clinically benign. To identify the molecular mechanism(s) responsible for the phenotypic differences, eight PFIC2 and two BRIC2...

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Share to ATP Binding Cassette Transporter, Subfamily B, Member 11
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Cholestasis, Intrahepatic

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Source

Identifiers

PMID: 17947449
DOI: 10.1152/ajpgi.00367.2007

Genes

ABCB11

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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