title: Autophagy, mitochondria and 3-nitropropionic acid joined in the same model.
abstract: Huntington's disease (HD) is a neurodegenerative disorder caused by a mutation in the gene encoding the huntingtin protein. Although the precise mechanism by which neuronal degeneration occurs is still unclear, several elements are important to its development: (1) altered gene expression and protein synthesis, (2) mitochondrial damage and (3) improper regulation of the autophagy programme. In this issue of British Journal of Pharmacology, Galindo and co-workers provide the first evidence for a role of the mitochondrial permeability transition pore (mPTP) in mitochondrial fragmentation and autophagy activation. In a model of cell death induced by 3-nitropropionic acid (3-NP) in human neural cells, the authors describe clear functions for mPTP and Bax, but not the mitochondrial fusion/fission machinery, mitochondrial fragmentation and autophagy (mitophagy). This commentary summarises the significance of this relationship and suggests several points for future development.
authors.affiliation: Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas, Departamento de Bioquímica y Biología Molecular y Genética, E. Enfermería y T.O., Universidad de Extremadura, Cáceres, Spain.
authors.forename: Rosa A
authors.initials: RA
authors.lastname: González-Polo
authors.forename: José M
authors.initials: JM
authors.lastname: Bravo-San Pedro
authors.forename: Rubén
authors.initials: R
authors.lastname: Gómez-Sánchez
authors.forename: Elisa
authors.initials: E
authors.lastname: Pizarro-Estrella
authors.forename: Mireia
authors.initials: M
authors.lastname: Niso-Santano
authors.forename: José M
authors.initials: JM
authors.lastname: Fuentes
mesh_terms: D000818:Animals; D001343:Autophagy / Q000187:drug effects*; D006801:Humans; D008297:Male; D008928:Mitochondria / Q000187:drug effects*; D033681:Mitochondrial Membrane Transport Proteins / Q000096:biosynthesis* / Q000187:drug effects*; D000083162:Mitochondrial Permeability Transition Pore; D009574:Nitro Compounds / Q000494:pharmacology*; D011422:Propionates / Q000494:pharmacology*
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D000083162:Mitochondrial Permeability Transition Pore; D009574:Nitro Compounds; D011422:Propionates; C015392:3-nitropropionic acid