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Phenotypes Associated with This Genotype
Genotype
MGI:4462847
Allelic
Composition
Gys2tm1.1Pro/Gys2tm1.1Pro
Tg(Alb-cre)21Mgn/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * C57BL/6J * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gys2tm1.1Pro mutation (0 available); any Gys2 mutation (13 available)
Tg(Alb-cre)21Mgn mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Decreased glycogen liver content in Gys2tm1.1Pro/Gys2tm1.1Pro Tg(Alb-cre)21Mgn/0 mice

homeostasis/metabolism
• fed mice exhibit reduced exercise capacity compared with control Gys2tm1.1Pro homozygotes
• during a fast, mice exhibit a more acute increase in ketone bodies than in similarly treated control Gys2tm1.1Pro homozygotes
• mice enter a fasted state quicker than similarly treated control Gys2tm1.1Pro homozygotes
• in fed and fasted states, mice exhibit reduced circulating glucose levels compared with similarly treated control Gys2tm1.1Pro homozygotes
• under fed condition
• mice exhibit enhanced gluconeogenesis compared with control Gys2tm1.1Pro homozygotes
• glycogen liver content is reduced 94% compared to in control Gys2tm1.1Pro homozygotes
• exercised mice exhibit a greater decrease in muscle glycogen levels compared with control Gys2tm1.1Pro homozygotes
• however, glycogen content in the skeletal muscle and kidney is normal
• fasting mice fail to exhibit a change in triglyceride levels unlike fasted control Gys2tm1.1Pro homozygotes
• at 7 and 15 months

cardiovascular system
• heart glucose uptake is lower than in control Gys2tm1.1Pro homozygotes

liver/biliary system
• at 7 and 15 months

muscle
• heart glucose uptake is lower than in control Gys2tm1.1Pro homozygotes

behavior/neurological
• fed mice exhibit reduced exercise capacity compared with control Gys2tm1.1Pro homozygotes

cellular
• heart glucose uptake is lower than in control Gys2tm1.1Pro homozygotes

Mouse Models of Human Disease
OMIM ID Ref(s)
Glycogen Storage Disease 0, Liver; GSD0A 240600 J:162043


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
07/12/2016
MGI 6.04
The Jackson Laboratory