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Phenotypes Associated with This Genotype
Genotype
MGI:5448463
Allelic
Composition
Tg(SOD1*H46R*H48Q)139Dbo/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J
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No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• by 4-6 months of age (J:119631)
• by 4-6 months of age (J:119631)

nervous system
• astrocytic reactions in the ventral midbrain, brain stem, and spinal cord (J:119631)
• astrocytic reactions in the ventral midbrain, brain stem, and spinal cord (J:119631)
• 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 (J:119631)
• 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 (J:119631)
• reduction in the numbers of large motor neurons in spinal cords of paralyzed mutants (J:119631)
• reduction in the numbers of large motor neurons in spinal cords of paralyzed mutants (J:119631)

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:119631


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