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Could successful (mitochondrial) networking help prevent Huntington's disease?

Lookup NU author(s): Dr Jorge Oliveira, Professor Robert Lightowlers

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Abstract

Polyglutamine expansions in huntingtin (Htt) are known to cause the profound neurodegenerative disorder, Huntington's disease (HD). Mitochondrial dysfunction has long been implicated in the pathophysiology of HD, but the underlying mechanism remains obscure. An article by Costa et al in this months edition describes a smooth mechanistic cascade from the well-accepted upstream event that mutant Htt is associated with Ca2+ handling abnormalities, through to apoptotic neuronal death. The proposed cascade implicates calcineurin, activated by abnormal Ca2+ levels, in the dephosphorylation of dynamin-1-like protein (Drp1.), increasing its association with mitochondria and promoting fission, cristae disruption, cytochrome c release and apoptosis (Fig 1). Together with the recent reports of increased mitochondrial fission in striatal neurons from HD patients, the article by Costa et al provides a compelling case for the role of abnormal mitochondrial networking in HD pathogenesis.


Publication metadata

Author(s): Oliveira JMA, Lightowlers RN

Publication type: Article

Publication status: Published

Journal: EMBO Molecular Medicine

Year: 2010

Volume: 2

Issue: 12

Pages: 487-489

Print publication date: 29/11/2010

ISSN (print): 1757-4676

ISSN (electronic): 1757-4684

Publisher: Wiley-Blackwell Publishing Ltd.

URL: http://dx.doi.org/10.1002/emmm.201000104

DOI: 10.1002/emmm.201000104


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