Mouse Genome Informatics
hm
    Ift88Tg737Rpw/Ift88Tg737Rpw
C3.FVB-Ift88Tg737Rpw
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
renal/urinary system
• Background Sensitivity: on a C3H background urine specific gravity and urine:serum osmolality ratio remain normal for much longer compared to mice on an FVB/N background
• Background Sensitivity: cysts develop at a slower rate on a C3H background compared to an FVB/N background (J:18508)
• proximal tubular cysts are seen at 15-20 days of age with a progressive shift in cyst localization to collecting ducts complete by 70 days of age (J:73583)
• at 20-30 days of age, cysts are about 0.58% of the total kidney area (J:73583)

liver/biliary system
• bilary dysplasia and hyperplasia are seen around the portal triad (J:73583)
• portal fibrosis (J:18508)
• Background Sensitivity: fibrosis is more severe on an inbred C3H background compared to an FVB/N background (J:73583)
• Background Sensitivity: unlike in mice on an inbred FVB/N, background proliferating oval cells are not seen
• Background Sensitivity: the liver lesion is less severe on a C3H background compared to an FVB/N background

endocrine/exocrine glands
• pancreatic ductular hyperplasia and dysplasia
• Background Sensitivity: pancreatic lesions develop at a much slower rate compared to mice on an FVB/N background
• bilary dysplasia and hyperplasia are seen around the portal triad (J:73583)

homeostasis/metabolism
• Background Sensitivity: increase is higher on an FVB/N background compared to a C3H background
• mild increase in serum bile acid concentrations
• Background Sensitivity: increase is higher on an FVB/N background compared to a C3H background
• Background Sensitivity: on a C3H background urine specific gravity and urine:serum osmolality ratio remain normal for much longer compared to mice on an FVB/N background

limbs/digits/tail
• Background Sensitivity: polydactyly is more variable on a C3H background compared to an FVB/N background

digestive/alimentary system
• pancreatic ductular hyperplasia and dysplasia
• Background Sensitivity: pancreatic lesions develop at a much slower rate compared to mice on an FVB/N background

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:18508 , J:73583