Mouse Genome Informatics
cn
    Gt(ROSA)26Sortm1(Kcnj11*V59M)Fmas/Gt(ROSA)26Sor+
Tg(Ins2-cre)23Herr/0

involves: 129S4/SvJae * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Reduction in islet insulin content in Gt(ROSA)26Sortm1(Kcnj11*V59M)Fmas/Gt(ROSA)26Sor+ Tg(Ins2-cre)23Herr/0 mice

endocrine/exocrine glands
• the percentage of islet area occupied by beta cells is somewhat decreased
• alpha cells are not confined to the islet mantle
• relative increase in the number of alpha cells
• some of the beta cells are irregularly shaped
• the percentage of beta cells is decreased
• reduction in islet insulin content by 5 weeks of age but not at 5 days of age
• K(sub)ATP currents in beta cells are larger compared to control cells an inhibition of these currents by glucose is impaired
• glucose induced calcium responses of beta cells are impaired
• impairment in basal and both first and second-phase glucose induced insulin secretion

homeostasis/metabolism
• impairment in basal and both first and second-phase glucose induced insulin secretion
• elevated glucose levels develop by P3
• overt diabetes develops by 5 weeks of age

growth/size/body
• seen in males by 4 to 6 weeks of age

renal/urinary system
• produce copious amounts of urine after when a few weeks old

Mouse Models of Human Disease
OMIM IDRef(s)
Diabetes Mellitus, Permanent Neonatal; PNDM 606176 J:144715