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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1*L126Z)#Deng
transgene insertion, Han-Xiang Deng
MGI:5449894
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0
involves: C57BL/6 * SJL MGI:5449903
tg2
Tg(SOD1*L126Z)#Deng/0 involves: C57BL/6 * SJL MGI:5449895


Genotype
MGI:5449903
cx1
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
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458




Genotype
MGI:5449895
tg2
Allelic
Composition
Tg(SOD1*L126Z)#Deng/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan is on average 359 days

nervous system
• mutants exhibit extensive protein aggregates of the mutant protein in the gray matter of the spinal cord at 350 days of age
• mutants develop an amyotrophic lateral sclerosis-like phenotype by the age of 300-400 days, with an average onset of 336.3 days

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458





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last database update
09/27/2016
MGI 6.05
The Jackson Laboratory