Background and gender effects on survival in the TgN (SOD1-G93A) 1Gur mouse model of ALS
TD Heiman-Patterson, JS Deitch… - Journal of the …, 2005 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder. While most
cases of ALS are sporadic, 10–15% are familial, and of these 15–20% possess a mutation in
the gene that codes for the enzyme Cu/Zn superoxide dismutase (SOD1). In families of ALS
patients with specific SOD1 mutations, affected members demonstrate significant
heterogeneity of disease and a large variation in age of onset and severity, suggesting that
there are genetic modifiers of disease expression. Transgenic mice expressing mutant forms …
cases of ALS are sporadic, 10–15% are familial, and of these 15–20% possess a mutation in
the gene that codes for the enzyme Cu/Zn superoxide dismutase (SOD1). In families of ALS
patients with specific SOD1 mutations, affected members demonstrate significant
heterogeneity of disease and a large variation in age of onset and severity, suggesting that
there are genetic modifiers of disease expression. Transgenic mice expressing mutant forms …