Phenotypes Associated with This Genotype

Genotype
MGI:5618431

• Allelic Composition: Abcb4^tm1Bor/Abcb4^tm1Bor
• Genetic Background: involves: 129P2/OlaHsd * BALB/c

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

immune system

bile duct inflammation ( J:168123 )

• mice develop sclerosing cholangitis

• propranolol treatment reduces inflammation

liver/biliary system

bile duct inflammation ( J:168123 )

• mice develop sclerosing cholangitis

• propranolol treatment reduces inflammation

liver fibrosis ( J:168123 )

• 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

decreased systemic arterial systolic blood pressure ( J:168123 )

• 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	J:168123