Mouse Genome Informatics
hm
    T(2;10)67Gso/T(2;10)67Gso
involves: 101 * C3H * C3H/Rl * C57BL/RlGso * SEC/RlGso
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• most of those that survive the first day of life die by 3-4 days of age
• many homozygotes die immediately after birth

renal/urinary system
• kidneys are bilaterally enlarged and pale
• elongated cysts that extend radially from the cortex towards the medullary pyramid
• collecting tubules within the cortex are elongated and dilated with cysts which are lined with cuboidal epithelium

liver/biliary system
• bile ducts are increased in number and dilated and no normal interlobular ducts are seen
• cysts are lined with a single layer of low columnar to cuboidal cells
• most homozygotes turn a yellow-orange color before death

growth/size/body
• homozytgotes are smaller than unaffected littermates at birth
• seen at 1-3 days of age

behavior/neurological
• homozytgotes are weaker than unaffected littermates at birth

endocrine/exocrine glands
• bile ducts are increased in number and dilated and no normal interlobular ducts are seen
• cysts are lined with a single layer of low columnar to cuboidal cells

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:75799