Mitochondrial dynamics and quality control in Huntington's disease

• Type: Article in International Scientific Journal
• Date: 2016-06-01

Authors

Pedro Guedes-Dias (Author) FFUP; Brigida Pinho (Author) FFUP; Tânia R. Soares (Author) FFUP; João de Proença (Author) FFUP; Michael R. Duchen (Author) Other; Jorge M A Oliveira (Author) FFUP

Journal

Title: Neurobiology of Disease

Vol. 90

Pages: 51-57

ISSN: 0969-9961

Publisher: Elsevier

Scientific classification

FOS: Medical and Health sciences > Health sciences

CORDIS: Health sciences > Neuroscience

Associated Projects

KDAC´S - Inibição de KDACs e dinâmica intracelular: impacto no desenvolvimento, sobrevivência e transmissão NEUROnal

Other information

DOI: 10.1016/j.nbd.2015.09.008

Abstract (EN): Huntington's disease (HD) is an inherited neurodegenerative disorder caused by polyglutamine expansion mutations in the huntingtin protein. Despite its ubiquitous distribution, expression of mutant huntingtin (mHtt) is particularly detrimental to medium spiny neurons within the striatum. Mitochondrial dysfunction has been associated with HD pathogenesis. Here we review the current evidence for mHtt-induced abnormalities in mitochondrial dynamics and quality control, with a particular focus on brain and neuronal data pertaining to striatal vulnerability. We address mHtt effects on mitochondrial biogenesis, protein import, complex assembly, fission and fusion, mitochondrial transport, and on the degradation of damaged mitochondria via autophagy (mitophagy). For an integrated perspective on potentially converging pathogenic mechanisms, we also address impaired autophagosomal transport and abnormal mHtt proteostasis in HD.

Language: English

Type (Professor's evaluation): Scientific