About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:5661390
Allelic
Composition
Agltm1Geno/Agltm1Geno
Genetic
Background
C57BL/6J-Agltm1Geno
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agltm1Geno mutation (0 available); any Agl mutation (7 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system

nervous system
• slight increase of glycogen content in brain frontal cortex, in the granular layer of the cerebellum adjacent to the Purkinje cell layer

mortality/aging
• increase in adult mortality within 1 year of age

behavior/neurological
• 8 month old mice perform less well on the accelerating rotarod than controls
• mice exhibit everted paws during walking resulting in a gait defect at 3 months of age
• mice exhibit significant performance variability on a treadmill with belt speed of 30 cm/s at 4 months of age and dramatic decrease in performance at 3 months of age and an inability to remain on the belt at 9 months of age
• mice however are able to execute the test with mild exercise for long distances at a belt speed of 15 cm/s for 350 m, although performance is worse than in controls

homeostasis/metabolism
• mice exhibit significant performance variability on a treadmill with belt speed of 30 cm/s at 4 months of age and dramatic decrease in performance at 3 months of age and an inability to remain on the belt at 9 months of age
• mice however are able to execute the test with mild exercise for long distances at a belt speed of 15 cm/s for 350 m, although performance is worse than in controls
• adult fed mice exhibit lower basal blood glucose levels
• however, serum triglycerides and total cholesterol levels are normal
• after 16 hours of fasting, glycemia is reduced in mutants compared to wild-type mice
• high levels in 3 and 12 month old fed mice; levels increase with age
• high levels in 3 and 12 month old fed mice; levels remain steadily higher
• high levels in 3 and 12 month old fed mice; levels increase with age
• serum CK is higher at 3 and 12 months of age
• increase in glycogen content in skeletal muscle including the diaphragm, liver, heart, and brain tissue of adults
• glycogen accumulation is seen in tongue tissue of 4 and 18 month old mice
• slight increase of glycogen content in brain frontal cortex, in the granular layer of the cerebellum adjacent to the Purkinje cell layer
• glycogen accumulation is seen in hepatocytes at 4, 8, 12, and 18 months of age
• glycogen in the liver has an abnormal structure
• glycogen granules are dispersed among the myofibrils in 4 month old muscles
• 12 month old muscle fibers contain large glycogen collections that are localized at the subsarcolemmal level and at intracytoplasmic sites and disrupt contractile units

liver/biliary system
• livers are dark red in color compared to lighter coloring in wild-type
• however, cirrhosis or adenomas are not seen and liver does not exhibit fibrosis, even in older mice
• glycogen accumulation is seen in hepatocytes at 4, 8, 12, and 18 months of age
• glycogen in the liver has an abnormal structure
• hepatocytes appear enlarged due to glycogen deposition
• 3 month old livers are increased to over 9% of total body weight and to 11% at 12 months of age, compared to 5 and 6% of total body weight in wild-type mice at these ages, respectively

muscle
• glycogen granules are dispersed among the myofibrils in 4 month old muscles
• 12 month old muscle fibers contain large glycogen collections that are localized at the subsarcolemmal level and at intracytoplasmic sites and disrupt contractile units

respiratory system
• older mice show an accelerated respiratory rate at basal conditions

skeleton
• seen in adults

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
glycogen storage disease III DOID:2748 OMIM:232400
J:218471


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)
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
03/31/2020
MGI 6.15
The Jackson Laboratory