Phenotypes Associated with This Genotype

Genotype
MGI:5286592

• Allelic Composition: Tg(SOD1*G127X)716Mrkl/Tg(SOD1*G127X)716Mrkl
• Genetic Background: B6JBom.Cg-Tg(SOD1*G127X)716Mrkl

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

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		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:175594