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Article

Morphologic findings in progressive familial intrahepatic cholestasis 2 (PFIC2): correlation with genetic and immunohistochemical studies.

The American journal of surgical pathology - 1 May 2011

Evason Kimberley, Bove Kevin E, Finegold Milton J, Knisely A S, Rhee Sue, Rosenthal Philip, Miethke Alexander G, Karpen Saul J, Ferrell Linda D, Kim Grace E

Abstract excerpt

Progressive familial intrahepatic cholestasis, type 2 (PFIC2), characterized by cholestasis in infancy that may progress to cirrhosis, is caused by mutation in ABCB11, which encodes bile salt export pump (BSEP). We correlated histopathologic, immunohistochemical, and ultrastructural features in PFIC2 with specific mutations and clinical course. Twelve patients with clinical PFIC2 and ABCB11 mutations were...

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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

Share to Child, Preschool

Share to Cholestasis, Intrahepatic

Share to Immunohistochemistry

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Share to Microscopy, Electron, Transmission

Source

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Identifiers

PMID: 21490445
DOI: 10.1097/PAS.0b013e318212ec87
PMCID: PMC3416050

Genes

ABCB11

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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