The groups were transgenic human wild-type SOD1 mice ([SOD1.sup.WT]), transgenic G93A
mice ([SOD1.sup.G93A]), and the age-matched nontransgenic controls.
However, in the aggressive G93A
disease model, there was no significant effect on disease progression or survival, but a significant delay in symptom onset was observed.
Muscle-restricted expression of a localized insulin-like growth factor-1 (Igf-1) isoform maintains muscle integrity and enhanced satellite cell activity in SOD1(G93A
) transgenic mice, inducing calcineurin-mediated regenerative pathways (36).
The genotype of the human mismatch repair gene hMLH1 at the G93A
locus was amplified by PCR with 0.4 [micro]mol/L each of the forward (5'-AGTAGCCGCTTCAGGGA-3') and reverse (5'-AGGACGGTGCGGTGAGAGTG-3') primers, as described previously (10).
Lab mice prone to sclerotic muscle atrophy (because of mutant protein SODI, Cu/Zn superoxide dismutase I, in their G93A
genes) hold much experimental promise.
Hamadeh, "Dietary Vitamin D 3 supplementation at 10x the adequate intake improves functional capacity in the G93A
transgenic mouse model of ALS, a pilot study," CNS Neuroscience & Therapeutics, vol.
Caloric restriction transiently improves motor performance but hastens clinical onset of disease in the Cu/Zn-superoxide dismutase mutant G93A
and A4V mutant human Cu, Zn-superoxide dismutase (hSOD1) gene-transfected motor neurons consistently display GSK-3[beta] hyperactivity along with inhibition of the PI3K/Akt pathway .
Andjus, "Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel expression in the brain of the amyotrophic lateral sclerosis SOD1(G93A
) rat model," Glia, vol.
GH gene fragments revealed five SNP loci (A12G, T45C, G84A, G93A
, and C133T) in BMs and TBs, but three SNP loci (A12G, T45C, and G93A
) in LWs.
Byers et al., "Effect of transgene copy number on survival in the G93A
SOD1 transgenic mouse model of ALS," Molecular Brain Research, vol.
de Vito et al., "Immune response in peripheral axons delays disease progression in SOD1(G93A
) mice," Journal of Neuroinflammation, vol.