Mouse Genome Informatics
cn
    Gys2tm1.1Pro/Gys2tm1.1Pro
Tg(Alb-cre)21Mgn/0

involves: 129S6/SvEvTac * C57BL/6 * C57BL/6J * DBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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
• in fed and fasted states, mice exhibit reduced circulating glucose levels compared with similarly treated control Gys2tm1.1Pro homozygotes
• mice enter a fasted state quicker than 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

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