Article
Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis.
Proceedings of the National Academy of Sciences of the United States of America - 19 Jan 2016
Proctor Elizabeth A, Fee Lanette, Tao Yazhong, Redler Rachel L, Fay James M, Zhang Yuliang, Lv Zhengjian, Mercer Ian P, Deshmukh Mohanish, Lyubchenko Yuri L, Dokholyan Nikolay V
Abstract excerpt
Since the linking of mutations in the Cu,Zn superoxide dismutase gene (sod1) to amyotrophic lateral sclerosis (ALS) in 1993, researchers have sought the connection between SOD1 and motor neuron death. Disease-linked mutations tend to destabilize the native dimeric structure of SOD1, and plaques containing misfolded and aggregated SOD1 have been found in the motor neurons of patients with ALS. Despite advances in...
Topics
- Amyotrophic Lateral Sclerosis
- Animals
- Cell Death
- Cell Line
- Enzyme Stability
- Humans
- Models, Biological
- Models, Molecular
- Motor Neurons
- Mutant Proteins