Amyotrophic lateral sclerosis associated with mutations in superoxide dismutase: A putative mechanism of degeneration

Brett M. Morrison, John Morrison

Research output: Contribution to journalReview article

73 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS) is a devastating neurologic disease that rapidly progresses from mild motor symptoms to severe motor paralysis and premature death. Until recently, there were few substantive studies conducted on the pathogenesis of the disease. With the genetic linkage of mutations in the superoxide dismutase (SOD-1) gene with familial ALS patients, new avenues for study have become available including transgenic mice and culture models. Although not yet providing a complete picture of the disease mechanism, studies utilizing these model systems have greatly advanced our understanding of the mechanism of degeneration and should eventually lead to putative therapeutic agents. In this review, we will present the important findings from these model systems, provide a framework in which to evaluate these findings, and speculate on the mechanism of degeneration initiated by the mutations in SOD-1.

Original languageEnglish (US)
Pages (from-to)121-135
Number of pages15
JournalBrain Research Reviews
Volume29
Issue number1
DOIs
StatePublished - Jan 1 1999
Externally publishedYes

Keywords

  • Excitotoxicity
  • Neurofilament
  • Transgenic

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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