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Phenotypes Associated with This Genotype
Genotype
MGI:5449903
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation (2 available)
Tg(SOD1*L126Z)#Deng mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan is on average 201.4 days

nervous system
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days

cellular
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 1 DOID:0060193 OMIM:105400
J:109458


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
04/18/2017
MGI 6.08
The Jackson Laboratory