Phenotypes Associated with This Genotype

Genotype
MGI:5428898

• Allelic Composition: Kras^tm4Tyj/Kras⁺; Trp53^tm1Brn/Trp53⁺; Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺
• Genetic Background: involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 * DBA

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

mortality/aging

premature death ( J:184949 )

• mean survival is 52 weeks of age

neoplasm

increased cholangiocarcinoma incidence ( J:184949 )

• mutants develop intrahepatic cholangiocarcinoma as early as 32 weeks of age

• intrahepatic cholangiocarcinoma recapitulates the histologic and molecular features of multistage progression of human intrahepatic cholangiocarcinoma

• intrahepatic cholangiocarcinoma is associated with intraductal papillary neoplasms of the bile ducts and Von Meyenburg complexes, or biliary hamartomas

• intrahepatic cholangiocarcinoma is characterized by elevated levels of autophagy

• different grades of differentiation are seen in the same tumor

increased metastatic potential ( J:184949 )

• tumors are highly metastatic, with tumors showing invasion into adjacent organs such as the diaphragm, bowel, pancreas and stomach or distant metastasis to the lymph nodes, spleen, lungs, or peritoneal cavity

increased hamartoma incidence ( J:184949 )

• intrahepatic cholangiocarcinoma is associated with biliary hamartomas

cardiovascular system

hemoperitoneum ( J:184949 )

• hepatic lesions frequently show hemorrhage into the peritoneal cavity and exhibit evidence of tumor necrosis

liver/biliary system

increased cholangiocarcinoma incidence ( J:184949 )

• mutants develop intrahepatic cholangiocarcinoma as early as 32 weeks of age

• intrahepatic cholangiocarcinoma recapitulates the histologic and molecular features of multistage progression of human intrahepatic cholangiocarcinoma

• intrahepatic cholangiocarcinoma is associated with intraductal papillary neoplasms of the bile ducts and Von Meyenburg complexes, or biliary hamartomas

• intrahepatic cholangiocarcinoma is characterized by elevated levels of autophagy

• different grades of differentiation are seen in the same tumor

endocrine/exocrine glands

increased cholangiocarcinoma incidence ( J:184949 )

• mutants develop intrahepatic cholangiocarcinoma as early as 32 weeks of age

• intrahepatic cholangiocarcinoma recapitulates the histologic and molecular features of multistage progression of human intrahepatic cholangiocarcinoma

• intrahepatic cholangiocarcinoma is associated with intraductal papillary neoplasms of the bile ducts and Von Meyenburg complexes, or biliary hamartomas

• intrahepatic cholangiocarcinoma is characterized by elevated levels of autophagy

• different grades of differentiation are seen in the same tumor

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
intrahepatic cholangiocarcinoma		DOID:4928		J:184949