mortality/aging
• many homozygotes die immediately after birth
• earliest onset in mice homozygous for this allele compared with mice homozygous for Bicc1jcpk or Bicc1jcpk-bpk
|
• most of those that survive the first day of life die by 3-4 days of age
|
behavior/neurological
endocrine/exocrine glands
• ducts are increased in number and dilated, extending into the hepatic lobes
|
• no normal interlobular ducts are seen
|
biliary cyst
(
J:75799
)
• 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
|
growth/size/body
biliary cyst
(
J:75799
)
• 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
|
• homozytgotes are smaller than unaffected littermates at birth
|
kidney cyst
(
J:75799
)
• narrow elongated cysts extend radially from the cortex toward the medullary pyramid
• cortical collecting tubules are elongated, lined with cuboidal epithelium and dilated by cysts
• collecting tubules and glomeruli seem compressed by cysts
|
• seen at 1-3 days of age
|
• bilateral enlargement
|
liver/biliary system
biliary cyst
(
J:75799
)
• 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 extreme in mice homozygous for this allele compared with mice homozygous for Bicc1jcpk or Bicc1jcpk-bpk
|
• ducts are increased in number and dilated, extending into the hepatic lobes
|
• normal parenchyma is lacking
|
• no normal interlobular ducts are seen
|
renal/urinary system
• pale and enlarged
|
kidney cyst
(
J:75799
)
• narrow elongated cysts extend radially from the cortex toward the medullary pyramid
• cortical collecting tubules are elongated, lined with cuboidal epithelium and dilated by cysts
• collecting tubules and glomeruli seem compressed by cysts
|
• bilateral enlargement
|