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Article

Generation of a bile salt export pump deficiency model using patient-specific induced pluripotent stem cell-derived hepatocyte-like cells.

Scientific reports - 2 Feb 2017

Imagawa Kazuo, Takayama Kazuo, Isoyama Shigemi, Tanikawa Ken, Shinkai Masato, Harada Kazuo, Tachibana Masashi, Sakurai Fuminori, Noguchi Emiko, Hirata Kazumasa, Kage Masayoshi, Kawabata Kenji, Sumazaki Ryo, Mizuguchi Hiroyuki

Abstract excerpt

Bile salt export pump (BSEP) plays an important role in hepatic secretion of bile acids and its deficiency results in severe cholestasis and liver failure. Mutation of the ABCB11 gene encoding BSEP induces BSEP deficiency and progressive familial intrahepatic cholestasis type 2 (PFIC2). Because liver transplantation remains standard treatment for PFIC2, the development of a novel therapeutic option is desired....

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Share to ATP Binding Cassette Transporter, Subfamily B, Member 11
Share to Bile Acids and Salts

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Cholestasis, Intrahepatic

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Source

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Identifiers

PMID: 28150711
DOI: 10.1038/srep41806
PMCID: PMC5288783

Genes

ABCB11

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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