Mouse Model: FVB.SMN2;Smn-, Severe Type I SMA

Brief Description: Mice that are homozygous for the targeted mutant Smn1 allele and carry the SMN2 transgene exhibit symptoms and neuropathology similar to patients afflicted with type I proximal spinal muscular atrophy (SMA). Mice that survive for several days exhibit decreased suckling and movement, labored breathing and trembling limbs after 48 hours. Stillborn mice and mice that succumb later are noticeably smaller than normal litermates. Muscle fibers (quadriceps and gastrocnemius) in affected mice display atrophy.

Genetic Background 

Allele Name: Transgene insertion 89 

Allele Type: Transgenic, (Humanized sequence, Hypomorph, Inserted expressed sequence)

Gene Symbol: Grm7, glutamate receptor, metabotropic 7

Site of Expression: Dendrites, axons, and soma of spinal motor neurons display distinct expression of GFPGFP expression mimics endogenous HLXB9 expression pattern. Fluorscence is detected in axons, dendrites, and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.

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