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Phenotypes Associated with This Genotype
Genotype
MGI:4413664
Allelic
Composition
Sco2tm1.1Easc/Sco2tm2.1Easc
Genetic
Background
129X1/SvJ-Sco2tm1.1Easc/Sco2tm2.1Easc
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sco2tm1.1Easc mutation (0 available); any Sco2 mutation (1 available)
Sco2tm2.1Easc mutation (0 available); any Sco2 mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• mice perform poorer than wild-type mice on a treadmill (J:155116)
• mice perform poorer than wild-type mice on a treadmill (J:155116)
• mitochondrial copper content is decreased in the heart and liver compared to in wild-type mice (J:155116)
• however, total amount of copper in organs examined is normal (J:155116)
• mitochondrial copper content is decreased in the heart and liver compared to in wild-type mice (J:155116)
• however, total amount of copper in organs examined is normal (J:155116)
• by organ wet weight (J:155116)
• by organ wet weight (J:155116)

muscle
• on a hanging wire test, mice display muscle weakness unlike wild-type mice (J:155116)
• male mice develop muscle weakness at 4 months while female mice develop muscle weakness at 8 months (J:155116)
• on a hanging wire test, mice display muscle weakness unlike wild-type mice (J:155116)
• male mice develop muscle weakness at 4 months while female mice develop muscle weakness at 8 months (J:155116)

liver/biliary system
• by organ wet weight (J:155116)
• by organ wet weight (J:155116)

behavior/neurological
• mice exhibit impaired motor performance on a standard treadmill compared with controls (J:176080)
• treatment with AICAR improves motor performance (J:176080)
• mice exhibit impaired motor performance on a standard treadmill compared with controls (J:176080)
• treatment with AICAR improves motor performance (J:176080)
• mice perform poorer than wild-type mice on a treadmill (J:155116)
• mice perform poorer than wild-type mice on a treadmill (J:155116)

cellular
• complex IV activities were reduced in all examined tissues from the Sco2tm1.1Easc/Sco2tm2.1Easc mice (by approximately 20-60%), with the lowest values in liver (by approximately 60%) (J:155116)
• reduced complex III activities in all tissues from the mutant mice (J:155116)
• complex IV activities were reduced in all examined tissues from the Sco2tm1.1Easc/Sco2tm2.1Easc mice (by approximately 20-60%), with the lowest values in liver (by approximately 60%) (J:155116)
• reduced complex III activities in all tissues from the mutant mice (J:155116)

Mouse Models of Human Disease
OMIM ID Ref(s)
Mitochondrial Complex IV Deficiency 220110 J:155116


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
02/02/2016
MGI 6.02
The Jackson Laboratory