Phenotypes Associated with This Genotype

Genotype
MGI:3813319

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * SJL

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

mortality/aging

premature death ( J:155806 )

• mean 266 day

nervous system

abnormal neuron morphology ( J:106420 )

• mice develop neurofilament-rich Lewy-body like inclusions in the perikarya and proximal axons of the spinal cord motorneurons at day 180 that increase with age but are not as prominent as in Tg(SOD1*G93A)1Gur mice

abnormal axonal transport ( J:106420 )

• at day 200, both fast and slow axonal transport are impaired in the ventral root compared to in wild-type axons

behavior/neurological

limb grasping ( J:106420 )

• develops at day 200

paralysis ( J:106420 , J:155806 )

• at day 250 (J:106420)

hindlimb paralysis ( J:155806 )

muscle

muscle weakness ( J:106420 )

• develops at day 200

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:106420