Mouse Genome Informatics
cn
    Jag1tm1Frad/Jag1tm1Frad
Tg(Tagln-cre)1Her/0

B6.Cg-Jag1tm1Frad Tg(Tagln-cre)1Her
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• most mice die within 2 days of birth with few surviving beyond 4 weeks
• most mice die within 2 days of birth with few surviving beyond 4 weeks

liver/biliary system
• multifoci
• absent or incompletely formed bile ducts near the portal veins
• epithelial cells fail to aggregate
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• accumulation of bile in liver parenchyma
• at birth

homeostasis/metabolism
• total and conjugated
• increased circulating gamma-glutamyl transferase

growth/size
• at birth and in mice that survive beyond 4 weeks, worsening over time

behavior/neurological
• in mice that survive beyond 4 weeks, worsening over time

cardiovascular system
• multifoci

integument
• in mice that survive beyond 4 weeks, worsening over time

endocrine/exocrine glands
• absent or incompletely formed bile ducts near the portal veins
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• epithelial cells fail to aggregate

Mouse Models of Human Disease
OMIM IDRef(s)
Alagille Syndrome 1; ALGS1 118450 J:166890