Article
The potential role of misfolded wild-type SOD1 protein in sporadic amyotrophic lateral sclerosis (ALS): a review of the evidence.
Neurobiology of disease - 1 Nov 2025
Marlow Thomas R, Bowden Katie M, Collins Mark O, Shaw Pamela J
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterised by the selective loss of motor neurons in the motor cortex, brainstem and spinal cord. In 1993, the first ALS-linked gene mutations were identified in the Cu,Zn superoxide dismutase (SOD1) gene, which account for approximately 20 % of familial ALS cases. The mechanism of toxicity in this subset of patients is thought to...
Topics
- Amyotrophic Lateral Sclerosis
- Humans
- Superoxide Dismutase-1
- Protein Folding
- Animals
- Motor Neurons
- Mutation
