Mouse Genome Informatics
hm1
    Bicc1jcpk-bpk/Bicc1jcpk-bpk
BALB/c-Bicc1jcpk-bpk
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• Background Sensitivity: lethality occurs earlier (14 - 21 days of age) on a mixed BALB/c and CAST/EiJ background
• die around 4 weeks of age from kidney failure

renal/urinary system
• occasional cystic dilation of Bowman's space involving about 6% of glomeruli is seen
• progressive kidney enlargement is detectable by 2 weeks of age (J:13276)
• cells lining the cysts become increasingly flattened with age and the number of mitoses in cyst-lining epithelial cells is increased (J:13276)
• at birth tubular dilation and early cyst formation is in the inner cortical zone and tubules undergo progressive cystic enlargement resulting in nephron destruction and the location of the cysts gradually shifts from the proximal tubules to the collecting ducts (J:13276)
• at birth cysts are seen in subcortical areas and at later stages cystic lesions are found predominantly in collecting tubular segments (J:21554)
• cysts are radially arrayed, a pattern typical of cysts in the collecting ducts and occasional cystic dilation of Bowman's space involving about 6% of glomeruli is seen (J:36348)
• Background Sensitivity: kidney cysts develop earlier on a mixed BALB/c and CAST/EiJ background (J:36348)

growth/size/body
• from massive enlargement of kidneys (J:21554)
• Background Sensitivity: distension is seen earlier (9 - 15 days of age) on a mixed BALB/c and CAST/EiJ background (J:36348)

behavior/neurological
• become increasingly lethargic after birth

homeostasis/metabolism
• progressive elevation in plasma urea levels with age 31+/-9 and 88+/-14 vs 18+/-1 and 24+/-4 in controls at day 15 and 25, respectively; however plasma bilirubin is similar to controls

liver/biliary system
• epithelial hyperplasia with a 50% increase in biliary epithelial cells by day 5, a 60% increase by day 15, and a 100% increase by day 25 on a per square mm basis
• the number of bile ducts per square mm is increased
• dilation of the cystic, hepatic, and common bile ducts is seen, always linked to the presence of kidney cysts; however, the gallbladdr was not dilated and had normal histology
• increase in the frequency of portal areas in which the portal vein is surrounded by irregularly shaped bile ducts and increased epithelial cell proliferation

endocrine/exocrine glands
• epithelial hyperplasia with a 50% increase in biliary epithelial cells by day 5, a 60% increase by day 15, and a 100% increase by day 25 on a per square mm basis
• the number of bile ducts per square mm is increased
• dilation of the cystic, hepatic, and common bile ducts is seen, always linked to the presence of kidney cysts; however, the gallbladdr was not dilated and had normal histology

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:13276 , J:21554 , J:36348


Mouse Genome Informatics
hm2
    Bicc1jcpk-bpk/Bicc1jcpk-bpk
involves: BALB/c * CAST/EiJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• die between 14 and 21 days of age
• Background Sensitivity: lethality occurs earlier compared to homozygotes on an inbred BALB/c background which die at 4 to 5 weeks of age

renal/urinary system
• Background Sensitivity: kidney cysts at 14 days of age are similar those in homozygotes on an inbred BALB/c background at 24 to 28 days of age

liver/biliary system
• gall bladder dilation is common
• liver parenchyma is pale

growth/size/body
• significantly runted shortly after birth
• detected at 9 to 15 days of age
• Background Sensitivity: distension was not seen on an inbred BALB/c background until after 2 weeks of age

endocrine/exocrine glands
• gall bladder dilation is common

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:36348


Mouse Genome Informatics
ht3
    Bicc1jcpk/Bicc1jcpk-bpk
involves: 101 * BALB/c * C3H * C57BL/6J * T STOCK
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
renal/urinary system
• small opalescent cysts seen at 11 to 17 days of age
• cysts are radially arrayed, a pattern typical of cysts in the collecting ducts and cystic dilation of Bowman's space involving about 17% of glomeruli, similar to Bicc1jcpk-bpk homozygotes

liver/biliary system
• tortuous ducts are generally lined with hyperplastic epithelium
• gall bladder dilation is common
• expanded portal tracts with multiple irregularly shaped and variably dilated bile ducts
• the portal triad is expanded by hematopoietic cells and immature stromal cells
• liver parenchyma is pale

growth/size/body
• visible by 7 to 10 days of age

endocrine/exocrine glands
• tortuous ducts are generally lined with hyperplastic epithelium
• gall bladder dilation is common

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:36348