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Phenotypes Associated with This Genotype
Genotype
MGI:5695480
Allelic
Composition
Agltm1a(EUCOMM)Wtsi/Agltm1a(EUCOMM)Wtsi
Genetic
Background
involves: C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agltm1a(EUCOMM)Wtsi mutation (2 available); any Agl mutation (7 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• severe glycogen accumulation in the heart muscle

behavior/neurological
• muscle strength of the forelimb and hind legs in the grip test are lower at 62 weeks of age
• reduction in motor activity at 1 year of age

homeostasis/metabolism
• severe glycogen accumulation in the heart muscle
• hypoglycemia in 3 week old mice
• blood glucose levels are reduced after 12 hours of fasting in 3 week old mice but not at 8 weeks of age
• however, triglycerides and total cholesterol are normal at 3 and 8 weeks of age after 12 hours of fasting
• both mice fed ad-lib and those with repeated fasting show increased serum alanine transaminase levels compared to controls
• liver transaminases further increase with age
• both mice fed ad-lib and those with repeated fasting show increased serum alkaline phosphatase levels compared to controls
• both mice fed ad-lib and those with repeated fasting show increased serum aspartate transaminase levels compared to controls
• after fasting, 7 week old mice show increased creatine phosphokinase levels
• however, creatine phosphokinase levels are normal in mice fed ad-lib
• severe glycogen accumulation in the liver
• severe glycogen accumulation in the diaphragm, quadriceps, and gastrocnemius muscles

liver/biliary system
• severe glycogen accumulation in the liver
• enlarged hepatocytes with pale cytoplasm at 4 weeks of age
• hepatomegaly in 4 week old mice
• progressive liver fibrosis after 4 weeks of age, with mild fibrosis and periportal fibrosis at 18 weeks of age and expended area of fibrosis by 28 weeks of age
• however, mice do not develop hepatic adenoma or carcinoma up to 40 weeks of age

mortality/aging
• with repeated fasting, 50% of mice die before 40 weeks of age

muscle
• severe glycogen accumulation in the heart muscle
• severe glycogen accumulation in the diaphragm, quadriceps, and gastrocnemius muscles
• 1 year old mice show signs of muscle weakness

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
glycogen storage disease III DOID:2748 OMIM:232400
J:212003


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
03/03/2020
MGI 6.15
The Jackson Laboratory