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Phenotypes Associated with This Genotype
Genotype
MGI:6268336
Allelic
Composition
Gcdhtm1Dmk/Gcdhtm1Dmk
Genetic
Background
involves: 129S4/SvJae * C57BL/6N * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gcdhtm1Dmk mutation (1 available); any Gcdh mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice show increased time to transverse the balance beam
• decrease in locomotor activity
• 4 of 5 mice show lethargic behavior after 24 hours on a high-lysine diet

growth/size/body
• 4 of 5 mice show critical weight loss of more than 15% 24 hours after the start of a 4.7% lysine diet

homeostasis/metabolism
• mice exhibit an increase in glutaric acid levels in the brain and liver
• glutarylcarnitine level is increased in the liver in mice fed a standard diet and increased in the brain and liver of mice fed a high-lysine diet
• lysine diet increases 2-aminoadipic acid (2-AA) levels more than in wild-type mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
glutaric acidemia I DOID:0111254 OMIM:231670
J:256227


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
02/16/2021
MGI 6.16
The Jackson Laboratory