Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency

Biochimica et Biophysica Acta - Bioenergetics

Volumn 1847, Issue 6-7, 2015, Pages 620-628

  Hickmann, Fernanda Hermes ^a   Cecatto, Cristiane ^a   Kleemann, Daniele ^a   Monteiro, Wagner Oliveira ^a   Castilho, Roger Frigério ^c   Amaral, Alexandre Umpierrez ^a   Wajner, Moacir ^a,b  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

^c UNIVERSITY OF CAMPINAS   (Brazil)

Author keywords

Calcium; dehydrogenase deficiency; Liver mitochondrial bioenergetics; Long chain 3 hydroxy acyl CoA; Mitochondrial permeability transition

Indexed keywords

3 HYDROXYPALMITIC ACID; 3 HYDROXYTETRADECANOIC ACID; ADENOSINE DIPHOSPHATE; CARBOXYLIC ACID; CYCLOSPORIN A; GLUTAMIC ACID; MALIC ACID; RUTHENIUM RED; SUCCINIC ACID; UNCLASSIFIED DRUG; 3 HYDROXYACYL COENZYME A DEHYDROGENASE; CALCIUM; FATTY ACID; NICOTINAMIDE ADENINE DINUCLEOTIDE PHOSPHATE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BIOENERGY; BRAIN MITOCHONDRION; CONTROLLED STUDY; ENZYME DEFICIENCY; LIVER MITOCHONDRION; LONG CHAIN 3 HYDROXY ACYL COA DEHYDROGENASE DEFICIENCY; METABOLIC INHIBITION; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRIAL PERMEABILITY; MITOCHONDRION SWELLING; NONHUMAN; OXYGEN CONSUMPTION; PRIORITY JOURNAL; RAT; ANIMAL; CARDIOMYOPATHY; CELL MEMBRANE PERMEABILITY; DEFICIENCY; DISORDERS OF LIPID AND LIPOPROTEIN METABOLISM; DRUG EFFECTS; ENERGY METABOLISM; METABOLISM; MITOCHONDRIAL MEMBRANE; MITOCHONDRIAL MYOPATHY; NEUROLOGIC DISEASE; OXIDATIVE PHOSPHORYLATION; PATHOLOGY; RHABDOMYOLYSIS; TRANSPORT AT THE CELLULAR LEVEL; WISTAR RAT;

RATTUS;

3-HYDROXYACYL COA DEHYDROGENASES; ANIMALS; BIOLOGICAL TRANSPORT; CALCIUM; CARDIOMYOPATHIES; CELL MEMBRANE PERMEABILITY; ENERGY METABOLISM; FATTY ACIDS; LIPID METABOLISM, INBORN ERRORS; MEMBRANE POTENTIAL, MITOCHONDRIAL; MITOCHONDRIA, LIVER; MITOCHONDRIAL MEMBRANES; MITOCHONDRIAL MYOPATHIES; MITOCHONDRIAL SWELLING; NADP; NERVOUS SYSTEM DISEASES; OXIDATIVE PHOSPHORYLATION; OXYGEN CONSUMPTION; RATS; RATS, WISTAR; RHABDOMYOLYSIS;

EID: 84928029397     PISSN: 00052728     EISSN: 18792650     Source Type: Journal    
DOI: 10.1016/j.bbabio.2015.04.003     Document Type: Article

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