Article
Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis.
The Journal of biological chemistry - 9 Apr 2004
Rakhit Rishi, Crow John P, Lepock James R, Kondejewski Leslie H, Cashman Neil R, Chakrabartty Avijit
Abstract excerpt
Proteinacious intracellular aggregates in motor neurons are a key feature of both sporadic and familial amyotrophic lateral sclerosis (ALS). These inclusion bodies are often immunoreactive for Cu,Zn-superoxide dismutase (SOD1) and are implicated in the pathology of ALS. On the basis of this and a similar clinical presentation of symptoms in the familial (fALS) and sporadic forms of ALS, we sought to investigate...
Topics
- Amyotrophic Lateral Sclerosis
- Anilino Naphthalenesulfonates
- Calorimetry
- Chromatography, Liquid
- Circular Dichroism
- Dose-Response Relationship, Drug
- Erythrocytes
- Fluorescent Dyes
- Humans
- Hydrogen-Ion Concentration