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

• Tipo: Outra Publicação em Revista Científica Internacional
• Data: 2010

Autores

Jorge M A Oliveira (Autor) FFUP; Robert N Lightowlers (Autor) Outra

Revista

Título: EMBO Molecular Medicine

Vol. 2

Páginas: 487-489

ISSN: 1757-4676

Editora: Wiley-Blackwell

Indexação

ISI Web of Knowledge - 11 Citações

Scopus - 12 Citações

Classificação Científica

FOS: Ciências médicas e da saúde > Outras ciências médicas

Outras Informações

ID Authenticus: 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.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 3