Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Abcb4-defect cholangitis mouse model with hydrophobic bile acid composition by in vivo liver-specific gene deletion

5 Aug 2024

Abstract excerpt

Progressive familial intrahepatic cholestasis (PFIC) is a liver disease that occurs during childhood and requires liver transplantation. ABCB4 is localized along the canalicular membranes of hepatocytes, transports phosphatidylcholine into bile, and its mutation causes PFIC3. Abcb4 gene-deficient mice established as animal models of PFIC3 exhibit cholestasis-induced liver injury. However, their phenotypes are...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Drug Transport and Resistance Mechanisms
Share to Pediatric Hepatobiliary Diseases and Treatments
Share to RNA Interference and Gene Delivery

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Source

Identifiers

PMID: 39111549
DOI: 10.1016/j.jlr.2024.100616

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 24 Oct 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Abcb4-defect cholangitis mouse model with hydrophobic bile acid composition by in vivo liver-specific gene deletionPMID 39111549Read ↗

Rapid in vivo evaluation system for cholestasis-related genes in mice with humanized bile acid profiles.Semantic proximity Read ↗

Gene therapy for progressive familial intrahepatic cholestasis type 3 in a clinically relevant mouse modelSemantic proximity Read ↗

Genetic Analysis of ABCB4 Mutations and Variants Related to the Pathogenesis and Pathophysiology of Low Phospholipid-Associated Cholelithiasis.Semantic proximity Read ↗

Gene Therapy for Acquired and Genetic CholestasisSemantic proximity Read ↗

Current and future therapies for inherited cholestatic liver diseases.Semantic proximity Read ↗

The bile salt export pump: clinical and experimental aspects of genetic and acquired cholestatic liver disease.Semantic proximity Read ↗

Inborn errors of biliary canalicular transport systems.Semantic proximity Read ↗

X-linked cholestasis in mouse due to mutations of the P4-ATPase ATP11C.Semantic proximity Read ↗

Synthetic human ABCB4 mRNA therapy rescues severe liver disease phenotype in a BALB/c.Abcb4-/- mouse model of PFIC3.Semantic proximity Read ↗

Canalicular ABC transporters and liver disease.Semantic proximity Read ↗

A Comprehensive Review of Progressive Familial Intrahepatic Cholestasis (PFIC): Genetic Disorders of Hepatocanalicular TransportersSemantic proximity Read ↗

Bile Acid Sequestration by Colesevelam Reduces Bile Acid Hydrophobicity and Improves Liver Pathology in <em>Cyp2c70<sup>-/-</sup></em> Mice with a Human-Like Bile Acid CompositionSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.