Article
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 9 Mar 2005
Vijayvergiya Chetan, Beal M Flint, Buck Jochen, Manfredi Giovanni
Abstract excerpt
An increasing body of evidence suggests that mitochondrial dysfunction plays an important role in the pathogenesis of familial amyotrophic lateral sclerosis associated with "gain of function" mutations in Cu/Zn superoxide dismutase 1 (SOD1). SOD1 is mostly a cytosolic protein, but a portion of SOD1 is localized in mitochondria of patients with familial amyotrophic lateral sclerosis and transgenic mouse models of...
Topics
- Amyotrophic Lateral Sclerosis
- Animals
- Brain
- Humans
- Mice
- Mice, Transgenic
- Mitochondria
- Mitochondrial Membranes
- Mutation
- Superoxide Dismutase
- Superoxide Dismutase-1