Mouse Genome Informatics
tg1
    Tg(SOD1*H46R*H48Q)139Dbo/0
involves: C3H/HeJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:119631