TY - GEN AU - Genin, Emmanuelle C AU - Plutino, Morgane AU - Bannwarth, Sylvie AU - Villa, Elodie AU - Cisneros-Barroso, Eugenia AU - Roy, Madhuparna AU - Ortega-Vila, Bernardo AU - Fragaki, Konstantina AU - Lespinasse, Francoise AU - Pinero-Martos, Estefania AU - Auge, Gaelle AU - Moore, David AU - Burte, Florence AU - Lacas-Gervais, Sandra AU - Kageyama, Yusuke AU - Itoh, Kie AU - Yu-Wai-Man, Patrick AU - Sesaki, Hiromi AU - Ricci, Jean-Ehrland AU - Vives-Bauza, Cristofol AU - Paquis-Flucklinger, Veronique PY - 2016 DO - 10.15252/emmm.201505496 SN - 1757-4676 UR - http://hdl.handle.net/20.500.12105/20246 AB - CHCHD10-related diseases include mitochondrial DNA instability disorder, frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) clinical spectrum, late-onset spinal motor neuropathy (SMAJ), and Charcot-Marie-Tooth disease type 2 (CMT2). Here,... LA - eng PB - Wiley KW - CHCHD10 KW - Mitochondria KW - Mitochondrial disease KW - Motor neuron disease KW - mtDNA instability KW - Genome, Mitochondrial KW - Oxidative Stress KW - Mitochondria KW - Alleles KW - Humans KW - Cell Line KW - HeLa Cells KW - Mitochondrial Proteins KW - Real-Time Polymerase Chain Reaction KW - Cytochromes c KW - Hydrogen Peroxide KW - Lysosomes KW - DNA, Mitochondrial KW - DNA Repair KW - Membrane Potential, Mitochondrial KW - Mutation KW - Apoptosis KW - Mitochondrial Diseases TI - CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis TY - research article ER -