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

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.

journal_name

Br J Pharmacol

authors

Muyderman H,Chen T

doi

10.1111/bph.12476

subject

Has Abstract

pub_date

2014-04-01 00:00:00

pages

2191-205

issue

8

eissn

0007-1188

issn

1476-5381

journal_volume

171

pub_type

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