Mouse Genome Informatics
tg
    Tg(SOD1*G93A)1Gur/0
B6.Cg-Tg(SOD1*G93A)1Gur
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mean survivals in male and female mice are 153 2 and 161 2 days respectively

growth/size/body
• decreased body weight starting at P65

behavior/neurological
• tremor onset occurred significantly sooner in male (912 days) compared to female (1002 days)
• deficits in rotarod test
• deficits in paw grip endurance test (PaGE)
• delayed chronic deficits in female compared to male
• decreased average locomotion velocity and rearing episodes
• significant motor deficits in male and female mice (at P45 and P65 respectively) in average locomotion velocity
• motor deficits in rearing episodes beginning at P45
• persist at each subsequent time point up to and beyond the onset of overt clinical symptoms
• deficits in all four open-field performance measures start earlier than the conventional PaGE or rotarod tests
• delayed chronic deficits in average velocity in female compared to male
• motor deficits in ambulatory distance and resting time in male mice beginning at P45
• transient or no deficits in ambulatory distance and rest behaviors, respectively in female

muscle
• smaller hindlimb calf girth dorsal/ventral (d/v) starting at P55
• smaller hindlimb calf girth medial/lateral starting at P65
• smaller hindlimb thigh girth medial/lateral starting at P75
• calf (d/v) and thigh muscle girth are able to predict genotype in pre-symptomatic animals with 80% or greater accuracy (about 40 day prior to tremor onset)

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:155140