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

• Type: Another Publication in an International Scientific Journal
• Date: 2010

Authors

Jorge M A Oliveira (Author) FFUP; Robert N Lightowlers (Author) Other

Journal

Title: EMBO Molecular Medicine

Vol. 2

Pages: 487-489

ISSN: 1757-4676

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 11 Citations

Scopus - 12 Citations

Scientific classification

FOS: Medical and Health sciences > Other medical sciences

Other information

Authenticus ID: P-003-0EK

DOI: 10.1002/emmm.201000104

Abstract (EN): 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 Ca(2+) handling abnormalities, through to apoptotic neuronal death. The proposed cascade implicates calcineurin, activated by abnormal Ca(2+) 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 3