Familial ALS: Apocynin extended the lives of mutant mice and reduced glial cell toxicity of cultured cells lines with a defective Superoxide Dismutase-1 (SOD1) gene -- a genetic defect found in some people with hereditary amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease).
Onset is typically after the age of 7 years and it is seen most frequently in the German shepherd dog, Pembroke Welsh corgi, and boxer dog, though the disorder is strongly associated with a gene mutation in SOD1 that has been found in 43 breeds as of 2008, including the wire fox terrier, Chesapeake Bay retriever, Rhodesian ridgeback, and Cardigan Welsh corgi.
One form of PBP is found to occur within patients that have a CuZn-superoxide dismutase (SOD1) mutation.
SOD1 |
Researchers believe the benefit derives from a newly discovered role for SOD1 as a self-regulating redox sensor for NADPH oxidase-derived O2• production.