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Phenotypes Associated with This Genotype
Genotype
MGI:5473683
Allelic
Composition
Coq9tm1.1Lcl/Coq9tm1.1Lcl
Genetic
Background
involves: C57BL/6 * C57BL/6NTac * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Coq9tm1.1Lcl mutation (0 available); any Coq9 mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all mice die between 3 and 6 months

nervous system
• caspase-independent
• in the brain white matter and medulla oblongata
• in the pons and medulla oblongata
• in the pons, cerebellum and encephalon
• loss of dendrites
• with neuronal death

behavior/neurological
• mildly at 3 months
• at 5 months
• at 3 and 5 months
• mild alterations in walking at 3 to 6 months
• rapid and progressive between 3 and 6 months

growth/size/body
• at 3 months
• at 3 and 6 months
• at 3 to 6 months

homeostasis/metabolism
• mice exhibit a reduction in coenzyme Q9 and Q10 in the cerebrum, cerebellum, heart, kidney, hind legs skeletal muscle and liver compared with wild-type mice

cardiovascular system

cellular
• caspase-independent
• reduced complex I, mitochondrial respiration and ATP synthesis in the brain
• oxidative damage in the brain

integument
• at P20 to P22, mice exhibit hair loss that is recovered in the next hair growth cycle

muscle
• encephalomyopathy

Mouse Models of Human Disease
OMIM ID Ref(s)
Coenzyme Q10 Deficiency, Primary, 5; COQ10D5 614654 J:193288


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
05/17/2016
MGI 6.03
The Jackson Laboratory