Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

• Published in: EMBO molecular medicine Vol. 2; no. 12; pp. 490 - 503
• Main Authors: Costa, Veronica, Giacomello, Marta, Hudec, Roman, Lopreiato, Raffaele, Ermak, Gennady, Lim, Dmitri, Malorni, Walter, Davies, Kelvin J. A., Carafoli, Ernesto, Scorrano, Luca
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.12.2010; WILEY‐VCH Verlag; EMBO Press; WILEY-VCH Verlag
• Subjects: Animals; Apoptosis; Calcineurin; Cell culture; Cell death; Cell Line; Cells, Cultured; Cristae; cristae remodelling; Cytochrome; Cytochrome c; Cytochromes c - metabolism; Dynamin; Dynamins; Experiments; Female; fission; Genes; Genetic analysis; Genomes; GTP Phosphohydrolases - genetics; GTP Phosphohydrolases - metabolism; Humans; Huntingtin; Huntington Disease - genetics; Huntington Disease - metabolism; Huntington Disease - physiopathology; Huntington's disease; Huntingtons disease; Hypersensitivity; Male; Mice; Microscopy, Electron, Transmission; Microtubule-Associated Proteins - genetics; Microtubule-Associated Proteins - metabolism; Mitochondria; Mitochondria - genetics; Mitochondria - physiology; Mitochondria - ultrastructure; Mitochondrial Proteins - genetics; Mitochondrial Proteins - metabolism; Models, Biological; Morphology; Mutation; Neurodegenerative diseases; Neurons - cytology; Neurons - metabolism; Pathogenesis; Phosphorylation; Polyglutamine diseases; Protein Transport; Proteins; Research Article; Software; Trinucleotide repeat diseases; apoptosis; Huntington's disease; cristae remodelling; mitochondria; fission

Huntington's disease (HD), a genetic neurodegenerative disease caused by a polyglutamine expansion in the Huntingtin (Htt) protein, is accompanied by multiple mitochondrial alterations. Here, we show that mitochondrial fragmentation and cristae alterations characterize cellular models of HD and...