Article
Polyglutamine spinocerebellar ataxias - from genes to potential treatments.
Nature reviews. Neuroscience - 1 Oct 2017
Paulson Henry L, Shakkottai Vikram G, Clark H Brent, Orr Harry T
Abstract excerpt
The dominantly inherited spinocerebellar ataxias (SCAs) are a large and diverse group of neurodegenerative diseases. The most prevalent SCAs (SCA1, SCA2, SCA3, SCA6 and SCA7) are caused by expansion of a glutamine-encoding CAG repeat in the affected gene. These SCAs represent a substantial portion of the polyglutamine neurodegenerative disorders and provide insight into this class of diseases as a whole. Recent...
Topics
- Animals
- Brain
- Disease Models, Animal
- Humans
- Models, Genetic
- Models, Neurological
- Mutation
- Nerve Tissue Proteins
- Peptides
- Spinocerebellar Ataxias