Mouse Genome Informatics
cx1
     Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0
involves: C57BL/6 * SJL
Key:
♀ phenotype observed in females WTSI Wellcome Trust Sanger Institute
♂ phenotype observed in males Euro Europhenome
N normal phenotype
mortality/aging
premature death ( J:109458 )
• lifespan is on average 201.4 days

nervous system
abnormal spinal cord grey matter morphology ( J:109458 )
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords
abnormal nervous system physiology ( J:109458 )
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days

cellular
abnormal mitochondrial crista morphology ( J:109458 )
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae

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


Mouse Genome Informatics
tg2
     Tg(SOD1*L126Z)#Deng/0
involves: C57BL/6 * SJL
Key:
♀ phenotype observed in females WTSI Wellcome Trust Sanger Institute
♂ phenotype observed in males Euro Europhenome
N normal phenotype
mortality/aging
premature death ( J:109458 )
• lifespan is on average 359 days

nervous system
abnormal spinal cord grey matter morphology ( J:109458 )
• mutants exhibit extensive protein aggregates of the mutant protein in the gray matter of the spinal cord at 350 days of age
abnormal nervous system physiology ( J:109458 )
• 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 IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458