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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Cox10tm1Ctm
targeted mutation 1, Carlos T Moraes
MGI:3605782
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Cox10tm1Ctm/Cox10tm1Ctm
Myl1tm1(cre)Sjb/Myl1+
involves: 129X1/SvJ * C57BL/6 MGI:3609951
cn2
Cox10tm1Ctm/Cox10tm1Ctm
Tg(Camk2a-cre)#Szi/0
involves: 129X1/SvJ * C57BL/6 * C57BL/6J * CBA MGI:5444474


Genotype
MGI:3609951
cn1
Allelic
Composition
Cox10tm1Ctm/Cox10tm1Ctm
Myl1tm1(cre)Sjb/Myl1+
Genetic
Background
involves: 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cox10tm1Ctm mutation (1 available); any Cox10 mutation (3 available)
Myl1tm1(cre)Sjb mutation (1 available); any Myl1 mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutant mice have a shorter life span (J:101747)
• females died younger than males (J:101747)
• mutant mice have a shorter life span (J:101747)
• females died younger than males (J:101747)

muscle
• showed areas of mitochondrial proliferation and markedly enlarged and swollen mitochondria (J:101747)
• no signs of fibrosis, inflammation, or apoptosis were seen (J:101747)
• showed areas of mitochondrial proliferation and markedly enlarged and swollen mitochondria (J:101747)
• no signs of fibrosis, inflammation, or apoptosis were seen (J:101747)
• mutant muscle fibers are more heterogeneous in size (J:101747)
• mutant muscle fibers are more heterogeneous in size (J:101747)
• at 3-4 months of age, severe reductions in muscle mass are seen (J:101747)
• at 3-4 months of age, severe reductions in muscle mass are seen (J:101747)
• maximal force evoked in mutant muscle is significantly smaller (J:101747)
• the muscles from mutant mice are more fatigable than control (J:101747)
• maximal force evoked in mutant muscle is significantly smaller (J:101747)
• the muscles from mutant mice are more fatigable than control (J:101747)
• mutant mice are healthy until approximately 3 months of age then develop a slowly progressive myopathy (J:101747)
• the myopathy starts earlier and worsens quicker in female (J:101747)
• mutant mice are healthy until approximately 3 months of age then develop a slowly progressive myopathy (J:101747)
• the myopathy starts earlier and worsens quicker in female (J:101747)

growth/size/body
• at 3-4 months of age, they started to lose weight (J:101747)
• female mutant mice weigh less at an early age (J:101747)
• at 3-4 months of age, they started to lose weight (J:101747)
• female mutant mice weigh less at an early age (J:101747)

skeleton
• starting at 3-4 months of age (J:101747)
• starting at 3-4 months of age (J:101747)

behavior/neurological
• starting at 3-4 months of age (J:101747)
• in treadmill performance test, 2-month old mutant mice had difficulty in performing the task (J:101747)
• the female performance was significantly poorer than males (J:101747)
• starting at 3-4 months of age (J:101747)
• in treadmill performance test, 2-month old mutant mice had difficulty in performing the task (J:101747)
• the female performance was significantly poorer than males (J:101747)

Mouse Models of Human Disease
OMIM ID Ref(s)
Mitochondrial Myopathy 251900 J:101747




Genotype
MGI:5444474
cn2
Allelic
Composition
Cox10tm1Ctm/Cox10tm1Ctm
Tg(Camk2a-cre)#Szi/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * C57BL/6J * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cox10tm1Ctm mutation (1 available); any Cox10 mutation (3 available)
Tg(Camk2a-cre)#Szi mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die between 8 and 12 months of age (J:188773)
• die between 8 and 12 months of age (J:188773)

growth/size/body
• mutants weight less than littermate controls after 4 and 5 months of age, for females and males, respectively (J:188773)
• mutants weight less than littermate controls after 4 and 5 months of age, for females and males, respectively (J:188773)

behavior/neurological
• decrease in rotarod performance first seen at 3 and 4 months of age for males and females, respectively (J:188773)
• decrease in rotarod performance first seen at 3 and 4 months of age for males and females, respectively (J:188773)
• at 3 months of age, mutants start to decrease their nocturnal activity and by 4 months, cycles of hyperactivity are seen and continue throughout their life span (J:188773)
• at 3 months of age, mutants start to decrease their nocturnal activity and by 4 months, cycles of hyperactivity are seen and continue throughout their life span (J:188773)

cellular
• mutants exhibit 80% of mitochondrial respiratory complex IV (CIV) activity of control levels in the cortex at 1 month of age and 33% of control levels by 4 months of age (J:188773)
• mutants exhibit decreased CIV activity in the hippocampus as well (J:188773)
• mutants exhibit 80% of mitochondrial respiratory complex IV (CIV) activity of control levels in the cortex at 1 month of age and 33% of control levels by 4 months of age (J:188773)
• mutants exhibit decreased CIV activity in the hippocampus as well (J:188773)

nervous system
• brain weight is normal at 1-4 months of age, however by 8 months of age, brains weigh about half of control weight (J:188773)
• brain weight is normal at 1-4 months of age, however by 8 months of age, brains weigh about half of control weight (J:188773)
• severe cortical atrophy by 8 months of age (J:188773)
• severe cortical atrophy by 8 months of age (J:188773)
• neuronal cell death in the cingulate cortex at 4 months of age, but not earlier (J:188773)
• neuronal cell death in the cingulate cortex at 4 months of age, but not earlier (J:188773)
• mutants show oxidative damage in the piriform cortex at 4 months of age and neuronal cell death at this time but not earlier (J:188773)
• mutants show oxidative damage in the piriform cortex at 4 months of age and neuronal cell death at this time but not earlier (J:188773)
• mutants exhibit progressive encephalopathy, with small lesions in the piriform cortex at 4 months of age, lesions in the striatum, outer cortical layers and hippocampus by 6 months of age, and massive degeneration of the cortex by 8 months of age (J:188773)
• mutants exhibit progressive encephalopathy, with small lesions in the piriform cortex at 4 months of age, lesions in the striatum, outer cortical layers and hippocampus by 6 months of age, and massive degeneration of the cortex by 8 months of age (J:188773)
• mutants consistently show neuronal cell death in the cingulate cortex, piriform cortex, and hippocampus/dentate gyrus at 4 months of age, but not earlier (J:188773)
• mutants consistently show neuronal cell death in the cingulate cortex, piriform cortex, and hippocampus/dentate gyrus at 4 months of age, but not earlier (J:188773)
• the hippocampus/dentate gyrus and CA1 region shows neuronal loss at 4 months of age (J:188773)
• the hippocampus/dentate gyrus and CA1 region shows neuronal loss at 4 months of age (J:188773)
• mutants show metabolic changes in the brain, with elevated levels of lactate and a decrease in n-acetyl aspartate (J:188773)
• mutants show metabolic changes in the brain, with elevated levels of lactate and a decrease in n-acetyl aspartate (J:188773)
• mutants exhibit a dramatic increase in GFAP immunoreactivity in the cortex and somewhat smaller increase in the hippocampus and piriform cortex at 4-5 months of age, indicating reactive glia (J:188773)
• mutants exhibit a dramatic increase in GFAP immunoreactivity in the cortex and somewhat smaller increase in the hippocampus and piriform cortex at 4-5 months of age, indicating reactive glia (J:188773)

homeostasis/metabolism
• mutants exhibit decreased mitochondrial respiratory complex IV enzymatic activity in the cortex and hippocampus (J:188773)
• mutants exhibit decreased mitochondrial respiratory complex IV enzymatic activity in the cortex and hippocampus (J:188773)

Mouse Models of Human Disease
OMIM ID Ref(s)
Mitochondrial Complex IV Deficiency 220110 J:188773





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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory