Phenotypes associated with this allele

• Allele Symbol: Tg(SOD1*G37R)1Dwc
• Allele Name: transgene insertion 1, Don W Cleveland
• Allele ID: MGI:3629226
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Isl1^tm1(cre)Tmj/Isl1^+ Tg(SOD1*G37R)1Dwc/0	involves: 129X1/SvJ * C57BL/6	MGI:3629232
cn2	Tg(ITGAM-cre)2781Gkl/0 Tg(SOD1*G37R)1Dwc/0	involves: C57BL/6 * CBA	MGI:3629233
tg3	Tg(SOD1*G37R)1Dwc/0	involves: C57BL/6	MGI:3629231

Genotype
MGI:3629232

cn1

• Allelic Composition: Isl1^tm1(cre)Tmj/Isl1⁺; Tg(SOD1*G37R)1Dwc/0
• Genetic Background: involves: 129X1/SvJ * C57BL/6

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

mortality/aging

premature death ( J:109131 )

• overall survival is extended 64 days

nervous system

axon degeneration ( J:109131 )

• in transgenic mice expressing a motorneuron specific Cre, disease onset is delayed 18 days

• progression from onset through early disease is delayed 31 days

• later disease progression is slowed with an average extension of 15 days

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109131

Genotype
MGI:3629233

cn2

• Allelic Composition: Tg(ITGAM-cre)2781Gkl/0; Tg(SOD1*G37R)1Dwc/0
• Genetic Background: involves: C57BL/6 * CBA

mortality/aging

premature death ( J:109131 )

• expression of Cre in microglia extends the lifespan of mutant SOD1-expressing mice by an average of 99 days compared to non-ITGAM-cre expressing mice

nervous system

axon degeneration ( J:109131 )

• progression of later stage disease is slowed by an average of 75 days in mice expressing both transgenes

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109131

Genotype
MGI:3629231

tg3

• Allelic Composition: Tg(SOD1*G37R)1Dwc/0
• Genetic Background: involves: C57BL/6

mortality/aging

premature death ( J:109131 )

• transgenic mice develop end-stage disease by 8.5-11 months of age

growth/size/body

weight loss ( J:109131 )

• mice display weight loss from denervation induced muscle atrophy

nervous system

decreased motor neuron number ( J:109131 )

• mice in end-stage disease display 55% spinal motor neuron death

motor neuron degeneration ( J:109131 )

• mice develop fatal progressive motorneuron disease