Phenotypes Associated with This Genotype

Genotype
MGI:4839171

• Allelic Composition: Tg(SOD1*G93A)1Gur/0
• Genetic Background: B6.Cg-Tg(SOD1*G93A)1Gur

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:155140 )

• mean survivals in male and female mice are 153 2 and 161 2 days respectively

growth/size/body

decreased body weight ( J:155140 )

• decreased body weight starting at P65

behavior/neurological

tremors ( J:155140 )

• tremor onset occurred significantly sooner in male (912 days) compared to female (1002 days)

impaired coordination ( J:155140 )

• deficits in rotarod test

decreased grip strength ( J:155140 )

• deficits in paw grip endurance test (PaGE)

• delayed chronic deficits in female compared to male

decreased vertical activity ( J:155140 )

• 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

decreased locomotor activity ( J:155140 )

• 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

decreased skeletal muscle size ( J:155140 )

• 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		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:155140