The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1863, Issue 1, 2017, Pages 284-291

  Frey, Samuel ^a   Geffroy, Guillaume ^a   Desquiret Dumas, Valerie ^a,b   Gueguen, Naig ^a,b   Bris, Celine ^a,b   Belal, Sophie ^a   Amati Bonneau, Patrizia ^a,b   Chevrollier, Arnaud ^a   Barth, Magalie ^b   Henrion, Daniel ^a   Lenaers, Guy ^a   Bonneau, Dominique ^a,b   Reynier, Pascal ^a,b   Procaccio, Vincent ^a,b  

^a UNIVERSITÉ D'ANGERS   (France)

^b ESA Angers   (France)

Author keywords

Complex I assembly; Ketone bodies; MELAS syndrome; Mitochondria; Mitochondrial diseases; Mitochondrial DNA

Indexed keywords

ADENOSINE TRIPHOSPHATE; GLUCOSE; KETONE BODY; LACTIC ACID; MITOCHONDRIAL DNA; NICOTINAMIDE ADENINE DINUCLEOTIDE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE);

ARTICLE; CONTROLLED STUDY; COPY NUMBER VARIATION; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DNA MUTATIONAL ANALYSIS; ENZYME ACTIVITY; ENZYME DEFECT; GLYCOLYSIS; HUMAN; HUMAN CELL; MELAS SYNDROME; MITOCHONDRIAL RESPIRATION; MUTANT; OXYGEN CONSUMPTION; PRIORITY JOURNAL; RESPIRATORY CHAIN; SYNTHESIS; CELL LINE; CELL RESPIRATION; COMPLICATION; DEFICIENCY; DRUG EFFECT; GENETICS; KETOGENIC DIET; METABOLISM; MITOCHONDRION; NERVE CELL; PATHOLOGY;

ADENOSINE TRIPHOSPHATE; CELL LINE; CELL RESPIRATION; DIET, KETOGENIC; DNA COPY NUMBER VARIATIONS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT COMPLEX I; HUMANS; KETONE BODIES; MELAS SYNDROME; MITOCHONDRIA; MITOCHONDRIAL DISEASES; NEURONS;

EID: 84995582883     PISSN: 09254439     EISSN: 1879260X     Source Type: Journal    
DOI: 10.1016/j.bbadis.2016.10.028     Document Type: Article

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