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Phenotypes Associated with This Genotype
Genotype
MGI:3775225
Allelic
Composition		 Smn1tm1Msd/Smn1tm1Msd
Tg(Prnp-SMN)92Ahmb/Tg(Prnp-SMN)92Ahmb
Grm7Tg(SMN2)89Ahmb/Grm7Tg(SMN2)89Ahmb
Genetic
Background		 involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grm7Tg(SMN2)89Ahmb mutation (34 available); any Grm7 mutation (125 available)
Smn1tm1Msd mutation (37 available); any Smn1 mutation (87 available)
Tg(Prnp-SMN)92Ahmb mutation (3 available)
♀ 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 Smn1tm1Msd 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 Smn1tm1Msd, 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

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
10/29/2024
MGI 6.24 		The Jackson Laboratory