Biotin deprivation impairs mitochondrial structure and function and has implications for inherited metabolic disorders

Molecular Genetics and Metabolism

Volumn 116, Issue 3, 2015, Pages 204-214

  Ochoa Ruiz, Estefanía ^a   Díaz Ruiz, Rodrigo ^a   Hernández Vázquez, Alaín de J ^a   Ibarra González, Isabel ^a   Ortiz Plata, Alma ^b   Rembao, Daniel ^b   Ortega Cuéllar, Daniel ^c   Viollet, Benoit ^d,e,f   Uribe Carvajal, Salvador ^a   Corella, José Ahmed ^a   Velázquez Arellano, Antonio ^a  

^a UNIVERSIDAD NACIONAL AUTÓNOMA DE MÉXICO   (Mexico)

^b NATIONAL INSTITUTE OF NEUROLOGY AND NEUROSURGERY   (Mexico)

^c INSTITUTO NACIONAL DE PEDIATRÍA   (Mexico)

^d INSTITUT COCHIN   (France)

^e CNRS   (France)

^f UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

Disrupted anaplerosis; Impaired oxidative phosphorylation; Inborn errors of metabolism; Mitochondrial damage and biotin deprivation

Indexed keywords

BIOTIN; CYTOCHROME C OXIDASE; HYPOXIA INDUCIBLE FACTOR 1ALPHA; INTERLEUKIN 6; PINK PROTEIN; PROTEIN; PROTEIN BNIP3; PYRUVATE CARBOXYLASE; STAT3 PROTEIN; UNCLASSIFIED DRUG; BIOTIN CARBOXYLASE; CARNITINE; LIGASE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOPHAGY; CARDIOMYOPATHY; CELL DENSITY; CELL ENERGY; CONTROLLED STUDY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENERGY METABOLISM; FIBROBLAST; GLUTEUS MAXIMUS MUSCLE; HEPG2 CELL LINE; MALE; METABOLIC DISORDER; METHYLMALONIC ACIDEMIA; MITOCHONDRION; MITOPHAGY; MOUSE; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PRIORITY JOURNAL; PROPIONIC ACIDEMIA; RAT; RESPIRATORY CHAIN; TRANSMISSION ELECTRON MICROSCOPY; ANIMAL; CELL CULTURE; CITRIC ACID CYCLE; DEFICIENCY; GENETICS; INBORN ERROR OF METABOLISM; KNOCKOUT MOUSE; METABOLISM; PATHOLOGY; ULTRASTRUCTURE;

ANIMALS; BIOTIN; CARBON-NITROGEN LIGASES; CARNITINE; CELLS, CULTURED; CITRIC ACID CYCLE; ELECTRON TRANSPORT COMPLEX IV; ENERGY METABOLISM; INTERLEUKIN-6; METABOLISM, INBORN ERRORS; MICE, KNOCKOUT; MITOCHONDRIA; MITOCHONDRIAL DEGRADATION; OXIDATIVE PHOSPHORYLATION; PYRUVATE CARBOXYLASE; RATS;

EID: 84948711997     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2015.08.009     Document Type: Article

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