title: Mitochondrial fragmentation in neuronal degeneration: Toward an understanding of HD striatal susceptibility.
abstract: Huntington's disease (HD) is an autosomal-dominant progressive neurodegenerative disorder that primarily affects medium spiny neurons within the striatum. HD is caused by inheritance of an expanded CAG repeat in the HTT gene, resulting in a mutant huntingtin (mHtt) protein containing extra glutamine residues. Despite the advances in understanding the molecular mechanisms involved in HD the preferential vulnerability of the striatum remains an intriguing question. This review discusses current knowledge that links altered mitochondrial dynamics with striatal susceptibility in HD. We also highlight how the modulation of mitochondrial function may constitute an attractive therapeutic approach to reduce mHtt-induced toxicity and therefore prevent the selective striatal neurodegeneration.
authors: Departament de Biomedicina, Facultat de Medicina, Universitat de Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Spain; Institut de Neurociències, Universitat de Barcelona, Barcelona, Spain.
authors: Marta
authors: M
authors: Cherubini
authors: Departament de Biomedicina, Facultat de Medicina, Universitat de Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Spain; Institut de Neurociències, Universitat de Barcelona, Barcelona, Spain. Electronic address: silviagines@ub.edu.
authors: Silvia
authors: S
authors: Ginés
mesh_terms: D000818:Animals; D003342:Corpus Striatum / Q000473:pathology*; D006801:Humans; D006816:Huntington Disease / Q000473:pathology*; D008928:Mitochondria / Q000473:pathology*; D009474:Neurons / Q000473:pathology*
keywords: Huntingtin; Mitochondria fission; Striatal vulnerability