Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: A possible role of mPTP opening as a pathomechanism in these disorders?

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1842, Issue 9, 2014, Pages 1658-1667

  Tonin, Anelise Miotti ^a,b   Amaral, Alexandre Umpierrez ^a   Busanello, Estela Natacha ^a   Gasparotto, Juciano ^a   Gelain, Daniel P ^a   Gregersen, Niels ^b   Wajner, Moacir ^a,c  

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

^b AARHUS UNIVERSITY HOSPITAL   (Denmark)

^c HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

Author keywords

Brain energy homeostasis; Calcium; Long chain 3 hydroxy acyl CoA dehydrogenase deficiency; Mitochondrial trifunctional protein deficiency; Permeability transition pore

Indexed keywords

1,2,3,6 TETRAHYDRO 1 METHYL 4 PHENYLPYRIDINE; ADENOSINE DIPHOSPHATE; CALCIUM ION; CARBON; CYCLOSPORIN A; CYTOCHROME C; DECANOIC ACID; HYDROGEN PEROXIDE; HYDROXY FATTY ACID; LONG CHAIN 3 HYDROXYACYL COENZYME A DEHYDROGENASE; MITOCHONDRIAL PERMEABILITY TRANSITION PORE; MITOCHONDRIAL TRIFUNCTIONAL PROTEIN; PALMITIC ACID; RUTHENIUM RED; 3 HYDROXYDODECANOIC ACID; 3 HYDROXYPALMITIC ACID; 3 HYDROXYTETRADECANOIC ACID; ACYL COENZYME A DEHYDROGENASE; ADENOSINE TRIPHOSPHATE; LONG CHAIN FATTY ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE PHOSPHATE; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BIOENERGY; BRAIN CORTEX; BRAIN DAMAGE; BRAIN MITOCHONDRION; CONTROLLED STUDY; ENZYME RELEASE; HOMEOSTASIS; MITOCHONDRION SWELLING; NEUROLOGIC DISEASE; NONHUMAN; PRIORITY JOURNAL; RAT; ANIMAL CELL; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRION;

BRAIN ENERGY HOMEOSTASIS; CALCIUM; LONG-CHAIN 3-HYDROXY ACYL-COA DEHYDROGENASE DEFICIENCY; MITOCHONDRIAL TRIFUNCTIONAL PROTEIN DEFICIENCY; PERMEABILITY TRANSITION PORE;

3-HYDROXYACYL COA DEHYDROGENASES; ACYL-COA DEHYDROGENASE, LONG-CHAIN; ADENOSINE TRIPHOSPHATE; ANIMALS; CALCIUM; CARDIOMYOPATHIES; CEREBRAL CORTEX; CYTOCHROMES C; ENERGY METABOLISM; HOMEOSTASIS; HYDROGEN PEROXIDE; LAURIC ACIDS; LIPID METABOLISM, INBORN ERRORS; MEMBRANE POTENTIAL, MITOCHONDRIAL; MITOCHONDRIA; MITOCHONDRIAL MEMBRANE TRANSPORT PROTEINS; MITOCHONDRIAL MYOPATHIES; MITOCHONDRIAL SWELLING; MITOCHONDRIAL TRIFUNCTIONAL PROTEIN; MYRISTIC ACIDS; NADP; NERVOUS SYSTEM DISEASES; OXIDANTS; PALMITIC ACIDS; RATS; RATS, WISTAR; RHABDOMYOLYSIS;

EID: 84904114430     PISSN: 09254439     EISSN: 1879260X     Source Type: Journal    
DOI: 10.1016/j.bbadis.2014.06.011     Document Type: Article

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