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Br J Pharmacol. 2014 Apr;171(8):2191-205. doi: 10.1111/bph.12476.

Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?

Author information

1
Centre for Neuroscience, Discipline of Medical Biochemistry, Flinders Medical Science and Technology, School of Medicine, Flinders University, Adelaide, SA, Australia.

Abstract

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by the selective death of upper and lower motor neurons which ultimately leads to paralysis and ultimately death. Pathological changes in ALS are closely associated with pronounced and progressive changes in mitochondrial morphology, bioenergetics and calcium homeostasis. Converging evidence suggests that impaired mitochondrial function could be pivotal in the rapid neurodegeneration of this condition. In this review, we provide an update of recent advances in understanding mitochondrial biology in the pathogenesis of ALS and highlight the therapeutic value of pharmacologically targeting mitochondrial biology to slow disease progression.

KEYWORDS:

SOD-1; TDP-43; amyotrophic lateral sclerosis; cell death; mitochondria; therapeutic

PMID:
24148000
PMCID:
PMC3976630
DOI:
10.1111/bph.12476
[Indexed for MEDLINE]
Free PMC Article

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