The Journal of Neuroscience, August 17, 2005, ():
Regular Exercise Prolongs Survival in a Type 2 Spinal Muscular Atrophy Model Mouse
J. Neurosci. Grondard et al.
25: 7615
Supplemental data
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Supplemental figure : Generation of type 2 SMA-like mice. (a). Mating scheme for generation of breeder animals giving only type 2 SMA-like mice. Females heterozygous for the knockout of the Smn locus and carrying only one copy of the SMN2 transgene (Smn+/-; SMN2) were bred with male Smn+/- mice. The SMA-like mice were classified as type 2, when survival ranged between 10 days and 4 weeks, according to Hsieh-Li et al. (2000). F1 is the first generation progeny. (b) Transgenic-mutant progeny from selected breeders. (c) The SMN expression profile was systematically investigated in the spinal cord of mice classified as type 2 SMA-like mice and used in this study. RT-PCR analysis of exons 4-8 of SMN transcripts showed that the exon 7-containing transcripts were dramatically decreased in spinal cords of type 2 SMA-like mice in comparison of (Smn+/-; SMN2) control mice. (d) Determination of SMN2 gene copy number using semi-quantitative multiplex PCR. Bold type characters referred to peak height (in arbitrary units) and lower case characters referred to molecular weight of PCR products (in bp) determined by electrophoretic migration.
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