Article
Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis.
Journal of neurochemistry - 1 Feb 1999
Bruening W, Roy J, Giasson B, Figlewicz D A, Mushynski W E, Durham H D
Abstract excerpt
Mutations in the Cu/Zn-superoxide dismutase (SOD-1) gene underlie some familial cases of amyotrophic lateral sclerosis, a neurodegenerative disorder characterized by loss of cortical, brainstem, and spinal motor neurons. We present evidence that SOD-1 mutants alter the activity of molecular chape...
Topics
- 3T3 Cells
- Amyotrophic Lateral Sclerosis
- Animals
- Cell Survival
- Chaperonins
- Gene Expression Regulation, Enzymologic
- Gene Transfer Techniques
- HSP70 Heat-Shock Proteins
- Heat-Shock Response
- Humans
- Mice
- Mice, Transgenic
- Motor Neurons
- Mutation
- Neuroprotective Agents