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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Bicc1jcpk
juvenile congenital polycystic kidney disease
MGI:1856920
Summary 5 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Bicc1jcpk/Bicc1jcpk involves: 101 * C3H * C57BL/6J * T STOCK MGI:3582933
hm2
 		Bicc1jcpk/Bicc1jcpk involves: 101 * C3H * T STOCK MGI:3582952
ht3
 		Bicc1jcpk/Bicc1+ involves: 101 * C3H * T STOCK MGI:3582953
ht4
 		Bicc1jcpk/Bicc1jcpk-bpk involves: 101 * BALB/c * C3H * C57BL/6J * T STOCK MGI:3582934
cx5
 		Bicc1jcpk/T(2;10)67Gso involves: 101 * C3H * C3H/Rl * C57BL/6 * C57BL/RlGso * SEC/RlGso MGI:3583140


Genotype
MGI:3582933
hm1
Allelic
Composition		 Bicc1jcpk/Bicc1jcpk
Genetic
Background		 involves: 101 * C3H * C57BL/6J * T STOCK
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicc1jcpk mutation (0 available); any Bicc1 mutation (65 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
renal/urinary system
kidney cortex cyst ( J:36348 )
• cysts in the proximal and distal tubules
increased glomerular capsule space ( J:36348 )
• dilation of Bowman's space in 95% of glomeruli

liver/biliary system
abnormal bile duct morphology ( J:36348 )
• branched bile ductules are lined with hyperplastic epithelium
abnormal portal triad morphology ( J:36348 )
• the portal triad is expanded by hematopoietic cells and immature stromal cells

endocrine/exocrine glands
abnormal bile duct morphology ( J:36348 )
• branched bile ductules are lined with hyperplastic epithelium

growth/size/body
kidney cortex cyst ( J:36348 )
• cysts in the proximal and distal tubules

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
autosomal recessive polycystic kidney disease DOID:0110861 J:36348




Genotype
MGI:3582952
hm2
Allelic
Composition		 Bicc1jcpk/Bicc1jcpk
Genetic
Background		 involves: 101 * C3H * T STOCK
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicc1jcpk mutation (0 available); any Bicc1 mutation (65 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
postnatal lethality, complete penetrance ( J:23047 , J:27506 )
• generally die between 7 to 10 days after birth with none surviving longer than 20 days (J:27506)

renal/urinary system
kidney cyst ( J:23047 , J:27506 )
• cysts are evident at birth and increase in number and size with age (J:23047)
• cysts are initially found in the inner cortical and medullary regions, by 4 days of age cysts have expanded to involve most areas of the kidney including the outer cortex, and by 7 days virtually the entire kidney is affected (J:23047)

liver/biliary system
enlarged gallbladder ( J:23047 )
• about 50% of homozygotes have an enlarged gallbladder

digestive/alimentary system
dilated pancreatic duct ( J:23047 )
• dilation of the pancreatic duct

growth/size/body
enlarged gallbladder ( J:23047 )
• about 50% of homozygotes have an enlarged gallbladder
kidney cyst ( J:23047 , J:27506 )
• cysts are evident at birth and increase in number and size with age (J:23047)
• cysts are initially found in the inner cortical and medullary regions, by 4 days of age cysts have expanded to involve most areas of the kidney including the outer cortex, and by 7 days virtually the entire kidney is affected (J:23047)
decreased body size ( J:27506 )
distended abdomen ( J:23047 )
• seen at 3 to 10 days of age

endocrine/exocrine glands
dilated pancreatic duct ( J:23047 )
• dilation of the pancreatic duct
enlarged gallbladder ( J:23047 )
• about 50% of homozygotes have an enlarged gallbladder

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
autosomal recessive polycystic kidney disease DOID:0110861 J:23047




Genotype
MGI:3582953
ht3
Allelic
Composition		 Bicc1jcpk/Bicc1+
Genetic
Background		 involves: 101 * C3H * T STOCK
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicc1jcpk mutation (0 available); any Bicc1 mutation (65 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
renal/urinary system
kidney cyst ( J:23047 )
• 4 out of 17 heterozygotes between 10 and 15 months of age display cyctic abnormalities in the glomeruli

growth/size/body
kidney cyst ( J:23047 )
• 4 out of 17 heterozygotes between 10 and 15 months of age display cyctic abnormalities in the glomeruli

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
autosomal dominant polycystic kidney disease DOID:898 J:23047




Genotype
MGI:3582934
ht4
Allelic
Composition		 Bicc1jcpk/Bicc1jcpk-bpk
Genetic
Background		 involves: 101 * BALB/c * C3H * C57BL/6J * T STOCK
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicc1jcpk mutation (0 available); any Bicc1 mutation (65 available)
Bicc1jcpk-bpk mutation (0 available); any Bicc1 mutation (65 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
renal/urinary system
kidney cyst ( J:36348 )
• 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
abnormal bile duct morphology ( J:36348 )
• tortuous ducts are generally lined with hyperplastic epithelium
dilated gallbladder ( J:36348 )
• gall bladder dilation is common
abnormal portal lobule morphology ( J:36348 )
• expanded portal tracts with multiple irregularly shaped and variably dilated bile ducts
abnormal portal triad morphology ( J:36348 )
• the portal triad is expanded by hematopoietic cells and immature stromal cells
pale liver ( J:36348 )
• liver parenchyma is pale

growth/size/body
kidney cyst ( J:36348 )
• 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
distended abdomen ( J:36348 )
• visible by 7 to 10 days of age
enlarged kidney ( J:36348 )

endocrine/exocrine glands
abnormal bile duct morphology ( J:36348 )
• tortuous ducts are generally lined with hyperplastic epithelium
dilated gallbladder ( J:36348 )
• gall bladder dilation is common

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
autosomal recessive polycystic kidney disease DOID:0110861 J:36348




Genotype
MGI:3583140
cx5
Allelic
Composition		 Bicc1jcpk/T(2;10)67Gso
Genetic
Background		 involves: 101 * C3H * C3H/Rl * C57BL/6 * C57BL/RlGso * SEC/RlGso
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicc1jcpk mutation (0 available); any Bicc1 mutation (65 available)
T(2;10)67Gso mutation (0 available); any T(2;10)67Gso mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
renal/urinary system
polycystic kidney ( J:75799 )
• polycystic kidneys seen by 10 days of age

growth/size/body
polycystic kidney ( J:75799 )
• polycystic kidneys seen by 10 days of age
distended abdomen ( J:75799 )
enlarged kidney ( J:75799 )




Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
12/10/2024
MGI 6.24 		The Jackson Laboratory