Phenotypes associated with this allele

• Allele Symbol: Tg(Prnp-SMN)92Ahmb
• Allele Name: transgene insertion 92, Arthur H M Burghes
• Allele ID: MGI:3774945
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Smn1^tm1Msd/Smn1^tm1Msd Tg(Prnp-SMN)92Ahmb/Tg(Prnp-SMN)92Ahmb Grm7^Tg(SMN2)89Ahmb/Grm7^Tg(SMN2)89Ahmb	involves: 129P2/OlaHsd * FVB/N	MGI:3775225
cx2	Smn1^tm1Msd/Smn1^tm1Msd Tg(Prnp-SMN)92Ahmb/0 Grm7^Tg(SMN2)89Ahmb/Grm7^Tg(SMN2)89Ahmb	involves: 129P2/OlaHsd * FVB/N	MGI:3775226
tg3	Tg(Prnp-SMN)92Ahmb/Tg(Prnp-SMN)92Ahmb	involves: FVB/N	MGI:3775197
tg4	Tg(Prnp-SMN)92Ahmb/0	involves: FVB/N	MGI:3775196

Genotype
MGI:3775225

cx1

• Allelic Composition: Smn1^tm1Msd/Smn1^tm1Msd; Tg(Prnp-SMN)92Ahmb/Tg(Prnp-SMN)92Ahmb; Grm7^{Tg(SMN2)89Ahmb}/Grm7^{Tg(SMN2)89Ahmb}
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

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

mortality/aging

premature death ( J:131663 )

• double mutants survive an average of 210 days, with many living more than 1 year

• lifespan is less than that of Smn1^tm1Msd heterozygotes

muscle

decreased skeletal muscle fiber size ( J:131663 )

• muscle fiber distribution is different from normal muscle; mice have a greater number of smaller diameter fibers relative to controls

nervous system

nervous system phenotype ( J:131663 )

N • mice have normal motor neuron counts and lumbar root counts, compared to triple homozygotes for Smn1^tm1Msd, Tg(ACTA1-SMN)63Ahmb, and Tg(SMN2)89Ahmb

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Werdnig-Hoffmann disease		DOID:13137	OMIM:253300	J:131663

Genotype
MGI:3775226

cx2

• Allelic Composition: Smn1^tm1Msd/Smn1^tm1Msd; Tg(Prnp-SMN)92Ahmb/0; Grm7^{Tg(SMN2)89Ahmb}/Grm7^{Tg(SMN2)89Ahmb}
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

mortality/aging

premature death ( J:131663 )

• double mutants survive an average of 150 days

Genotype
MGI:3775197

tg3

• Allelic Composition: Tg(Prnp-SMN)92Ahmb/Tg(Prnp-SMN)92Ahmb
• Genetic Background: involves: FVB/N

normal phenotype

no abnormal phenotype detected ( J:131663 )

• expression of the transgene does not adversely affect the phenotype relative to wild-type

Genotype
MGI:3775196

tg4

• Allelic Composition: Tg(Prnp-SMN)92Ahmb/0
• Genetic Background: involves: FVB/N

normal phenotype

no abnormal phenotype detected ( J:131663 )

• expression of the transgene does not adversely affect the phenotype relative to wild-type