Phenotypes Associated with This Genotype

Genotype
MGI:3843203

• Allelic Composition: Men1^tm1Ctre/Men1^tm1Ctre; Tg(Pdx1-cre)89.1Dam/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * CBA * FVB/N

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

mortality/aging

premature death ( J:146440 )

• 50% of mice are dead by 14 months of age unlike wild-type mice

endocrine/exocrine glands

abnormal pancreatic islet morphology ( J:146440 )

• at 3 and 12 months, the vasculature of some islets is abnormal with dilation of blood vessels and intense tortuosity unlike in wild-type mice

• however, treatment with sunitinib decreases in islet vascularity

enlarged pancreatic islets ( J:146440 )

• as early as 5 to 6 months

pancreatic islet hyperplasia ( J:146440 )

• as early as 5 to 6 months

increased insulinoma incidence ( J:146440 )

• by 10 to 12 months of age, mice develop insulinoma that are filled with blood islands or lacunae unlike wild-type mice

• however, mice do not develop gastrinoma unlike in other MEN1 models

abnormal pancreas physiology ( J:146440 )

• mice exhibit more proliferating cells within their islets compared with wild-type mice

• however, proliferation of exocrine cells is normal and treatment with sunitinib decreases islet cell proliferation

homeostasis/metabolism

decreased circulating glucose level ( J:146440 )

• at 10 months of age

increased circulating insulin level ( J:146440 )

• starting at 5 months of age

neoplasm

increased insulinoma incidence ( J:146440 )

• by 10 to 12 months of age, mice develop insulinoma that are filled with blood islands or lacunae unlike wild-type mice

• however, mice do not develop gastrinoma unlike in other MEN1 models

cardiovascular system

abnormal blood vessel morphology ( J:146440 )

• at 3 and 12 months, the vasculature of some islets is abnormal with dilation of blood vessels and intense tortuosity unlike in wild-type mice

• however, treatment with sunitinib decreases in islet vascularity

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
multiple endocrine neoplasia type 1		DOID:10017	OMIM:131100	J:146440