Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acid β-oxidation intermediates on mitochondrial oxidative phosphorylation

Journal of Inherited Metabolic Disease

Volumn 19, Issue 2, 1996, Pages 161-164

  Ventura, F V ^a,b   Ruiter, J P N ^a   Ijlst, L ^a   De Almeida, I Tavares ^b   Wanders, R J A ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

^b UNIVERSITY OF LISBON   (Portugal)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A; ACYLCARNITINE; ADENOSINE TRIPHOSPHATE; DICARBOXYLIC ACID; LONG CHAIN FATTY ACID; MALIC ACID; PALMITOYL COENZYME A; UNCLASSIFIED DRUG;

CONFERENCE PAPER; CONTROLLED STUDY; ENZYME PHOSPHORYLATION; FATTY ACID OXIDATION; FIBROBLAST CULTURE; HUMAN; HUMAN CELL; LACTIC ACIDOSIS; MITOCHONDRIAL RESPIRATION; SKIN FIBROBLAST;

EID: 0029925716     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF01799419     Document Type: Conference Paper

References (7)

1. Duran M, Wanders RJA, de Jager JP, et al (1991) 3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment. Eur J Pediatr 150: 190-195.
2. Pande SV, Blanchaer MC (1971) Reversible inhibition of mitochondrial adenosine diphosphate phosphorylation by long-chain acyl-coenzyme A esters. J Biol Chem 246: 402-411.
3. Treem WR, Rinaldo P, Hale DE, et al (1994) Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Hepatology 19: 339-345.
4. Ventura FV, Ruiter JPN, Ijlst L, Almeida IT, Wanders RJA (1995) Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: Implications for long-chain fatty acid β-oxidation disorders. Biochim Biophys Acta 1272: 14-20.
5. Wanders RJA, van Roermund CWT, van Wijland MJA, et al (1987) Peroxisomal fatty acid β-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders. J Clin Invest 80: 1778-1783.
6. Wanders RJA, Ruiter JPN, Wijburg FA (1993) Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies. Biochim Biophys Acta 1181: 219-222.
7. Williamson JR, Corkey BE (1969) Methods Enzymol 13: 434-513.