Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below

Mechanism and treatment of motoneuron degeneration in ALS: what have SOD1 mutants told us?

Author information

  • Department of Pharmacology, University of Massachusetts Medical School, Worcester 01655, USA. zuoshang.xu@umassmed.edu

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes motoneuron degeneration, skeletal muscle atrophy, paralysis and death. The identification of mutations in Cu,Zn superoxide dismutase (SOD1) as a genetic cause of this disease has led to the creation of a number of in-vitro and in-vivo models. Experiments have been carried out in these model systems to address fundamental questions related to the disease: (1) what is the nature of toxicity of the mutated SOD1? (2) what are the cellular targets and pathways that lead to neuronal degeneration? (3) what makes motoneurons particularly vulnerable to the toxicity of the mutant enzyme? and (4) are there effective treatments for ALS based on current hypotheses regarding the disease mechanism? Current research on these questions is reviewed.

PMID:
11465016
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Loading ...
    Write to the Help Desk