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Phenotypes Associated with This Genotype
involves: 129P2/OlaHsd * BALB/c
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Abcb4tm1Bor mutation (1 available); any Abcb4 mutation (37 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• mice develop sclerosing cholangitis
• propranolol treatment reduces inflammation

liver/biliary system
• mice develop sclerosing cholangitis
• propranolol treatment reduces inflammation
• mice develop periportal fibrosis after 3 months of age, characterized by onion skin-like matrix formation around the bile ducts
• after 5 months, septal formation and massive matrix deposition is seen around ducts in the liver and at 8 months, periportal fibrosis is frequently accompanied by septae formation and prominent proliferation of the bile ducts
• propranolol treatment improves liver architecture and reduces inflammation and fibrosis progression

cardiovascular system
• mice treated with propranolol long-term show a minor reduction in the average systolic blood pressure without impact on viability

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
primary sclerosing cholangitis DOID:0060643 OMIM:613806

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
MGI 6.22
The Jackson Laboratory