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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1*H46R*H48Q)139Dbo
transgene insertion 139, David Borschelt
MGI:3695885
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(SOD1*H46R*H48Q)139Dbo/0 involves: C3H/HeJ * C57BL/6J MGI:5448463


Genotype
MGI:5448463
tg1
Allelic
Composition
Tg(SOD1*H46R*H48Q)139Dbo/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*H46R*H48Q)139Dbo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• by 4-6 months of age

nervous system
• astrocytic reactions in the ventral midbrain, brain stem, and spinal cord
• numerous ubiquitin-immunoreactive, Thioflavin-S positive fibrillar protein aggregates resembling hyaline inclusions are seen in the spinal cord, ventral midbrain, and the brain stem, with fewer in the cerebellum
• reduction in the numbers of large motor neurons in spinal cords of paralyzed mutants

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 1 DOID:0060193 OMIM:105400
J:119631





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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