Title | Superoxide dismutase and the death of motoneurons in ALS. |
Publication Type | Journal Article |
Year of Publication | 2001 |
Authors | Beckman JS, Estévez AG, Crow JP, Barbeito L |
Journal | Trends Neurosci |
Volume | 24 |
Issue | 11 Suppl |
Pagination | S15-20 |
Date Published | 2001 Nov |
ISSN | 0166-2236 |
Keywords | Amyotrophic Lateral Sclerosis, Cell Death, Humans, Motor Neurons, Superoxide Dismutase |
Abstract | Amyotrophic lateral sclerosis (ALS) is a lethal disease that is characterized by the relentless death of motoneurons. Mutations to Cu-Zn superoxide dismutase (SOD), though occurring in just 2-3% of individuals with ALS, remain the only proven cause of the disease. These mutations structurally weaken SOD, which indirectly decreases its affinity for Zn. Zn-deficient SOD induces apoptosis in motoneurons through a mechanism involving peroxynitrite. Importantly, Zn-deficient wild-type SOD is just as toxic as Zn-deficient ALS mutant SOD, suggesting that the loss of Zn from wild-type SOD could be involved in the other 98% of cases of ALS. Zn-deficient SOD could therefore be an important therapeutic target in all forms of ALS. |
Alternate Journal | Trends Neurosci. |
PubMed ID | 11881740 |