Spinal Muscular Atrophy Mouse Model Resource

Use this resource to identify and select the most appropriate mouse model of SMA for your research and to obtain a guide to help you efficiently work with these mice.

• See a complete list of JAX® Mice for spinal muscular atrophy research

Comparison of Featured SMA Models

This table summarizes the differences among important mouse models for SMA to help you easily find the right strain for your research:

Featured JAX® Mice Models of SMA

FVB.Cg-Grm7^{Tg(SMN2)89Ahmb} Smn1^tm1Msd/J

(005024)

• Common name: Burghes’ Severe Model
• Genetic background: FVB/N; incipient congenic (N5)
• Double mutant contains a knockout of mouse Smn1 (caused by insertion of a lacZ reporter) and a transgene with one normal copy of human SMN2 (Monani et al. 2000)
• Affected mice exhibit abnormal muscular innervation, motor neuron degeneration, tremors, abnormal suckling behavior
• Mortality: stillborn or death by postnatal days 4-6

FVB.Cg-Grm7^{Tg(SMN2)89Ahmb} Smn1^tm1Msd Tg(SMN2*delta7)4299Ahmb/J

(005025)

• Common name: SMNdelta7 or Moderate Type II SMA mice
• Genetic background: FVB/N; incipient congenic (N6)
• Triple mutant contains a knockout of mouse Smn1 (caused by insertion of a lacZ reporter) and two transgenes, one with a single normal copy of human SMN2 and the second with a human SMN2 promoter regulating expression of human SMN2 cDNA that lacks exon 7 (SMNdelta7) (Le et al. 2005)
• Affected mice exhibit abnormal muscular innervation and neuromuscular synapse morphology, impaired righting response by postnatal day 5 (P5), abnormal gate and impaired balance and limb coordination by P10, and skeletal muscle fiber atrophy by P14
• Mortality: death by postnatal days 15-22 (mean survival of 17.7 days)

FVB.Cg-Smn1^tm1Hung Tg(SMN2)2Hung/J

(005058)

• Common name: SMA-like mice line 2
• Genetic background: FVB/N; fully congenic (N10)
• Double mutant containing a knockout (deletion of exon 7) in mouse Smn1 and a transgene expressing normal human SMN2 (Hsieh-Li et al. 2000)
• Double homozygotes have thickened tails and otherwise normal phenotype
• Mice homozygous for Smn1^tm1Hung and hemizygous for Tg(SMN2)2Hung exhibit abnormal motor neuron morphology, decreased motor neuron number, muscular atrophy, and hindlimb paralysis
• Mortality in mice homozygous for Smn1^tm1Hung and hemizygous for Tg(SMN2)2Hung: death by postnatal days 15-16

FVB.129(B6)-Smn1^{tm5(Smn1/SMN2)Mrph}/J

(008604)

• Common name: Smn1^C
• Genetic background: FVB/NJ (001800); fully congenic using speed congenic protocol (N8)
• Expresses a targeted knock-in mutation containing two coding sequences; the first generates a mouse:human hybrid protein in which mouse Smn1 exons 7 and 8 were replaced by human exons 7 and 8, and the second is a full copy of the human SMN2 gene (Osborne et al. 2012)
• Homozygotes exhibit tail necrosis, low body weight, diminished grip strength, and lower bone mineral content and density with age
• Normal life span