Phenotypes Associated with This Genotype

Genotype
MGI:3583451

• Allelic Composition: Ift88^Tg737Rpw/Ift88^Tg737Rpw
• Genetic Background: C3.FVB-Ift88^Tg737Rpw

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

renal/urinary system

kidney cyst ( J:18508 , J:73583 )

• 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)

decreased urine osmolality ( J:73583 )

• 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

liver/biliary system

abnormal bile duct morphology ( J:18508 , J:73583 )

• bilary dysplasia and hyperplasia are seen around the portal triad (J:73583)

liver fibrosis ( J:18508 , 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)

absent oval cells ( J:73583 )

• Background Sensitivity: unlike in mice on an inbred FVB/N, background proliferating oval cells are not seen

abnormal oval cell physiology ( J:18508 )

• Background Sensitivity: the liver lesion is less severe on a C3H background compared to an FVB/N background

endocrine/exocrine glands

abnormal pancreatic duct morphology ( J:73583 )

• pancreatic ductular hyperplasia and dysplasia

• Background Sensitivity: pancreatic lesions develop at a much slower rate compared to mice on an FVB/N background

abnormal bile duct morphology ( J:18508 , J:73583 )

• bilary dysplasia and hyperplasia are seen around the portal triad (J:73583)

homeostasis/metabolism

increased circulating alkaline phosphatase level ( J:73583 )

• Background Sensitivity: increase is higher on an FVB/N background compared to a C3H background

increased bile salt level ( J:73583 )

• mild increase in serum bile acid concentrations

• Background Sensitivity: increase is higher on an FVB/N background compared to a C3H background

decreased urine osmolality ( J:73583 )

• 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

polydactyly ( J:18508 )

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

digestive/alimentary system

abnormal pancreatic duct morphology ( J:73583 )

• pancreatic ductular hyperplasia and dysplasia

• Background Sensitivity: pancreatic lesions develop at a much slower rate compared to mice on an FVB/N background

growth/size/body

kidney cyst ( J:18508 , J:73583 )

• 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)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive polycystic kidney disease		DOID:0110861		J:18508 , J:73583