mortality/aging

premature death ( J:175594 )

• mean survival is 250 days

behavior/neurological

paralysis ( J:175594 )

• 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

forelimb paralysis ( J:175594 )

• about 1/3 show forelimb onset rather than hindlimb onset of paralysis

hindlimb paralysis ( J:175594 )

nervous system

abnormal astrocyte morphology ( J:175594 )

• develop Lewy body like hyaline inclusions in astrocytes

astrocytosis ( J:175594 )

abnormal motor neuron morphology ( J:175594 )

• develop Lewy body like hyaline inclusions in motor neurons

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