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

Hakan Muyderman, Tong Chen

    Research output: Contribution to journalReview articlepeer-review

    56 Citations (Scopus)

    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.

    Original languageEnglish
    Pages (from-to)2191-2205
    Number of pages15
    JournalBritish Journal of Pharmacology
    Volume171
    Issue number8
    DOIs
    Publication statusPublished - Apr 2014

    Keywords

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

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