Mouse Genome Informatics
tg
    Tg(SOD1*G127X)716Mrkl/Tg(SOD1*G127X)716Mrkl
B6JBom.Cg-Tg(SOD1*G127X)716Mrkl
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mean survival is 250 days

behavior/neurological
• disease progression takes about 7-10 days from the time of first sign compared to about 3 weeks in mice carrying Tg(SOD1*G93A)1Gur
• about 1/3 show forelimb onset rather than hindlimb onset of paralysis

nervous system
• develop Lewy body like hyaline inclusions in astrocytes
• develop Lewy body like hyaline inclusions in motor neurons

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:175594