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Phenotypes Associated with This Genotype
involves: 129X1/SvJ * C57BL/6J * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Ins2-cre)23Herr mutation (1 available)
Wfs1tm1Perm mutation (0 available); any Wfs1 mutation (22 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• at 24 weeks of age, but not at 16, have lower weights

• both fasting and fed glucose:insulin ratios are elevated
• develop diabetes
• impaired glucose-stimulated insulin secretion
• progressive development of hyperglycemia
• fed glucose concentrations are higher at 24 weeks of age, but mean fasted glucose concentrations are normal
• fasting insulin levels are significantly reduced at 12 weeks of age and fed insulin levels are reduced at 12 and 24 weeks of age
• progressive glucose intolerance, with mild glucose intolerance 60 min after glucose injection by 12 weeks of age and significant glucose intolerance 30, 60, and 120 min after glucose injection by 16 weeks of age

endocrine/exocrine glands
• 2.5-fold increase in caspase 3-positive nuclei in islets, indicating increased apoptosis
• reduction of beta cell mass is seen at 24 weeks of age
• reduction of the beta cell:pancreas ratio is seen at 24 weeks of age
• asymmetry and disruption of islet architecture is apparent at 12 weeks of age, with an alteration of the ratio of beta to non-beta cells within the islet
• secretory granules are reduced in beta cells and majority of cells exhibit abundant dilated ER, indicating increased ER stress in beta cells
• impaired glucose-stimulated insulin secretion

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Wolfram syndrome 1 DOID:0110629 OMIM:222300

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.08
The Jackson Laboratory