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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 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Cox10tm1Ctm/Cox10tm1Ctm
Slc6a3tm1.1(cre)Bkmn/?
B6.Cg-Cox10tm1Ctm Slc6a3tm1.1(cre)Bkmn MGI:5775427
cn2
Cox10tm1Ctm/Cox10tm1Ctm
Myl1tm1(cre)Sjb/Myl1+
involves: 129X1/SvJ * C57BL/6 MGI:3609951
cn3
Cox10tm1Ctm/Cox10tm1Ctm
Tg(Camk2a-cre)#Szi/0
involves: 129X1/SvJ * C57BL/6 * C57BL/6J * CBA MGI:5444474


Genotype
MGI:5775427
cn1
Allelic
Composition
Cox10tm1Ctm/Cox10tm1Ctm
Slc6a3tm1.1(cre)Bkmn/?
Genetic
Background
B6.Cg-Cox10tm1Ctm Slc6a3tm1.1(cre)Bkmn
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cox10tm1Ctm mutation (1 available); any Cox10 mutation (8 available)
Slc6a3tm1.1(cre)Bkmn mutation (1 available); any Slc6a3 mutation (38 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice exhibit a decline in motor activity and coordination by 2 months of age, maximum decline is reached by 4 months of age
• mice are not able to perform the pole test by 2 months of age, however, treatment with high dose L-DOPA (25 mg/kg) or pioglitazone improves performance
• impaired rotarod performance by 2 months of age, however, treatment with high dose L-DOPA (25 mg/kg) or pioglitazone improves performance
• walking time is decreased on rotarod at 4 and 6 months as compared to younger animals
• decrease in rearing activity by 2 months of age, maximum decline is reached by 4 months of age
• mice move less during nocturnal cycle and in open field test (distance travelled) as compared to controls; treatment with high dose L-DOPA (25 mg/kg) results in hyperactivity and stereotypic movements
• maximum decline in motor performance is reached at 4 months of age

hematopoietic system
• activation of microglial cells in midbrain as detected by Iba1 immunoreactivity
• treatment with pioglitazone for 4 months results in reduction in Iba1 immunoreactivity in midbrain

growth/size/body
• pups are born at a lower body weight than controls and remain at a lower weight throughout life
• administration of pioglitazone increases weight to control levels

immune system
• activation of microglial cells in midbrain as detected by Iba1 immunoreactivity
• treatment with pioglitazone for 4 months results in reduction in Iba1 immunoreactivity in midbrain
• CXCL10 and CCL2 are increased in striatum
• in midbrain, levels of IL6, IL10, CXCL10 and CCL2 trend upward, but do not reach significance
• increase in IL1B in midbrain in 2 month old mice
• IL1B is slightly increased in striatum but does not reach significance

homeostasis/metabolism
• decrease in dopamine and dopamine metabolites (to a lesser extent) in striatum by 2 months of age as compared to controls
• dopamine depletion continues through out life but does not worsen

nervous system
• activation of microglial cells in midbrain as detected by Iba1 immunoreactivity
• treatment with pioglitazone for 4 months results in reduction in Iba1 immunoreactivity in midbrain
• decrease in dopamine and dopamine metabolites (to a lesser extent) in striatum by 2 months of age as compared to controls
• dopamine depletion continues through out life but does not worsen
• substantial loss of dopaminergic cell bodies in the substantial nigra as measured by reduction of tyrosine hydroxylase (TH) and dopamine transporter (DAT) content
• widespread axonal degeneration in striatum

Mouse Models of Human Disease
OMIM ID Ref(s)
Parkinson Disease, Late-Onset; PD 168600 J:231810




Genotype
MGI:3609951
cn2
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 (8 available)
Myl1tm1(cre)Sjb mutation (1 available); any Myl1 mutation (5 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutant mice have a shorter life span
• females died younger than males

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

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

skeleton
• starting at 3-4 months of age

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

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




Genotype
MGI:5444474
cn3
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 (8 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

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

behavior/neurological
• decrease in rotarod performance first seen at 3 and 4 months of age for males and females, respectively
• 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

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
• mutants exhibit decreased CIV activity in the hippocampus as well

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
• severe cortical atrophy by 8 months of age
• neuronal cell death in the cingulate cortex at 4 months of age, but not earlier
• mutants show oxidative damage in the piriform cortex at 4 months of age and neuronal cell death at this time but not earlier
• 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
• mutants consistently show neuronal cell death in the cingulate cortex, piriform cortex, and hippocampus/dentate gyrus at 4 months of age, but not earlier
• the hippocampus/dentate gyrus and CA1 region shows neuronal loss at 4 months of age
• mutants show metabolic changes in the brain, with elevated levels of lactate and a decrease in n-acetyl aspartate
• 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

homeostasis/metabolism
• mutants exhibit decreased mitochondrial respiratory complex IV enzymatic activity in the cortex and hippocampus

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





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory