SOD1 and mitochondria in ALS: A dangerous liaison

Maria Teresa Carrì, Mauro Cozzolino

Research output: Contribution to journalArticlepeer-review

Abstract

Mutant Cu,Zn superoxide dismutase (mutSOD1) is found in a subset of patients with familial amyotrophic lateral sclerosis (ALS), a fatal progressive paralysis due to loss of motor neurons. In the present article, we review existing evidence linking the expression of mutSOD1 to the many facets of mitochondrial dysfunction in ALS, with a focus on recent studies suggesting that the association and misfolding of the mutant protein (and possibly of the wild type protein as well) within these organelles is causally linked to their functional and structural alterations. Energy deficit, calcium mishandling and oxidative stress are paralleled by alteration in mitochondrial motility, dynamics and turnover and most probably lead to mitochondriadependent cell death. Thus, the development of new, selective mitochondria-targeted therapies may constitute a promising approach in the treatment of SOD1-linked ALS.

Original languageEnglish
Pages (from-to)593-599
Number of pages7
JournalJournal of Bioenergetics and Biomembranes
Volume43
Issue number6
DOIs
Publication statusPublished - Dec 2011

Keywords

  • Amyotrophic lateral sclerosis
  • Mitochondria
  • Motor neuron
  • SOD1

ASJC Scopus subject areas

  • Physiology
  • Cell Biology

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