Mitochondrial involvement in amyotrophic lateral sclerosis

Trigger or target?

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

Despite numerous reports demonstrating mitochondrial abnormalities associated with amyotrophic lateral sclerosis (ALS), the role of mitochondrial dysfunction in the disease onset and progression remains unknown. The intrinsic mitochondrial apoptotic program is activated in the central nervous system of mouse models of ALS harboring mutant superoxide dismutase 1 protein. This is associated with the release of cytochrome-c from the mitochondrial intermembrane space and mitochondrial swelling. However, it is unclear if the observed mitochondrial changes are caused by the decreasing cellular viability or if these changes precede and actually trigger apoptosis. This article discusses the current evidence for mitochondrial involvement in familial and sporadic ALS and concludes that mitochondria is likely to be both a trigger and a target in ALS and that their demise is a critical step in the motor neuron death.

Original languageEnglish
Pages (from-to)113-131
Number of pages19
JournalMolecular Neurobiology
Volume33
Issue number2
DOIs
StatePublished - May 31 2006

Fingerprint

Amyotrophic Lateral Sclerosis
Neurologic Mutant Mice
Mitochondrial Swelling
Motor Neurons
Cytochromes c
Disease Progression
Mitochondria
Central Nervous System
Apoptosis
Proteins
Amyotrophic lateral sclerosis 1

Keywords

  • ALS
  • Apoptosis
  • Mitochondria
  • SOD1

ASJC Scopus subject areas

  • Neuroscience(all)
  • Physiology

Cite this

Mitochondrial involvement in amyotrophic lateral sclerosis : Trigger or target? / Bacman, Sandra R.; Bradley, Walter G; Moraes, Carlos T.

In: Molecular Neurobiology, Vol. 33, No. 2, 31.05.2006, p. 113-131.

Research output: Contribution to journalArticle

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