Phenotypes Associated with This Genotype

Genotype
MGI:4430413

• Allelic Composition: Gt(ROSA)26Sor^{tm1(CAG-Kcnj11*,-GFP)Nich}/Gt(ROSA)26Sor⁺; Tg(Ins2-cre)23Herr/0
• Genetic Background: involves: C57BL/6 * CBA/J

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

growth/size/body

slow postnatal weight gain ( J:146650 )

• body weight of neonates is normal

• growth retardation after birth

• body weight is 50% of controls by three months

• pancreas weight/body weight ratio is normal

homeostasis/metabolism

decreased insulin secretion ( J:146650 )

• by 6 weeks of age loss of response to injected glucose

abnormal circulating glucose level ( J:146650 )

• dramatic lowering of glucose levels after insulin injection

hyperglycemia ( J:146650 )

• blood glucose is over 200mg/dl within 2 weeks of birth

• progressively increases to over 600mg/dl by 2 months

abnormal circulating glucagon level ( J:146650 )

decreased circulating glucagon level ( J:146650 )

• levels become undetectable as diabetes becomes more severe

increased circulating glucagon level ( J:146650 )

• significantly elevated early in diabetes development

decreased circulating insulin level ( J:146650 )

• plasma insulin levels decrease with time

endocrine/exocrine glands

abnormal pancreas morphology ( J:146650 )

abnormal pancreatic islet morphology ( J:146650 )

abnormal pancreatic alpha cell morphology ( J:146650 )

• alpha cells infiltrate center of islets after the onset of diabetes

decreased pancreatic alpha cell number ( J:146650 )

• are lost as diabetes becomes more severe

decreased pancreatic beta cell number ( J:146650 )

• beta cells are lost as diabetes develops

decreased pancreatic islet number ( J:146650 )

• number of islet is reduced by 50% early in diabetes and by more than 90% later

small pancreatic islets ( J:146650 )

• islet mass decreases to about 10% of controls

decreased pancreas weight ( J:146650 )

• pancreas weight relative to controls becomes reduced over time

• pancreas weight/body weight ratio is normal

abnormal pancreatic beta cell physiology ( J:146650 )

• insulin content of pancreas drops to about 10% of control levels

decreased insulin secretion ( J:146650 )

• by 6 weeks of age loss of response to injected glucose

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
permanent neonatal diabetes mellitus		DOID:0060639	OMIM:606176	J:146650