Título: Neurobiology of DiseaseImportada do Authenticus Pesquisar Publicações da Revista

Vol. 90

Páginas: 51-57

ISSN: 0969-9961

Editora: Elsevier

FOS: Ciências médicas e da saúde > Ciências da saúde

CORDIS: Ciências da Saúde > Neurociências

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.

Idioma: Inglês

Tipo (Avaliação Docente): Científica