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

dc.contributor.authorMuyderman, H.
dc.contributor.authorChen, T.
dc.date.issued2014
dc.description.abstractAmyotrophic 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.
dc.description.statementofresponsibilityH Muyderman and T Chen
dc.identifier.citationBritish Journal of Pharmacology, 2014; 171(8):2191-2205
dc.identifier.doi10.1111/bph.12476
dc.identifier.issn0007-1188
dc.identifier.issn1476-5381
dc.identifier.orcidChen, T. [0000-0002-7681-9632]
dc.identifier.urihttp://hdl.handle.net/2440/106788
dc.language.isoen
dc.publisherWiley-Blackwell
dc.relation.granthttp://purl.org/au-research/grants/nhmrc/1023780
dc.rights© 2013 The British Pharmacological Society
dc.source.urihttps://doi.org/10.1111/bph.12476
dc.subjectAmyotrophic lateral sclerosis; mitochondria; SOD-1; TDP-43; therapeutic; cell death
dc.titleMitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?
dc.typeJournal article
pubs.publication-statusPublished

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