Mouse Genome Informatics
tg
    Tg(SOD1*)DF7Yaw/0
C57BL/6-Tg(SOD1*)DF7Yaw
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mean age of death is 372 +/- 78 days

behavior/neurological
• mutants first show signs of hindlimb paraparesis and relatively symmetrical forelimb weakness (hemiparesis)

nervous system
• reactive gliosis in the spinal cord, predominantly in the lower spinal cord
• eosinophilic cytoplasmic inclusions are seen in the motor neurons that remain; inclusions resemble Lewy body-like hyaline inclusions, with the halo of inclusions composed of neurofilamentous structure and the core of granule-associated fibrils
• mean age of onset of motor neuron disease symptoms is 337 +/- 101 days
• loss of anterior horn cells in the spinal cord, predominantly in the lower spinal cord

muscle
• muscle atrophy is seen at the end stage of disease
• some mutants exhibit muscle cramps after exercise as a first symptom of disease

skeleton

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:97932