Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs onmitochondrial energy metabolism

Biochimica et Biophysica Acta - Bioenergetics

Volumn 1777, Issue 10, 2008, Pages 1276-1282

  Sauer, Sven Wolfgang ^a   Okun, Juergen G ^a   Hoffmann, Georg F ^a   Koelker, Stefan ^a   Morath, Marina A ^a  

^a UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

Author keywords

Acyl CoAs; Acylcarnitine; Alpha ketoglutarate dehydrogenase complex; Complex III; Mitochondrial energy metabolism; Octanoyl CoA; Organic acid; Pyruvate dehydrogenase complex; Respiratory chain

Indexed keywords

ACYL COENZYME A; ACYLCARNITINE; CARBOXYLIC ACID; CYTOCHROME C OXIDASE; OXOGLUTARATE DEHYDROGENASE; PYRUVATE DEHYDROGENASE COMPLEX; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINATE DEHYDROGENASE (UBIQUINONE); UBIQUINOL CYTOCHROME C REDUCTASE;

ABSORPTION SPECTROSCOPY; ACIDURIA; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; ENERGY METABOLISM; ENZYME DEFICIENCY; ENZYME INHIBITION; FATTY ACID OXIDATION; JAMAICAN VOMITING SICKNESS; MITOCHONDRION; NONHUMAN; PATHOGENESIS; PATHOPHYSIOLOGY; PRIORITY JOURNAL; RESPIRATORY CHAIN; REYE SYNDROME; VOMITING;

ACIDS; ACYL COENZYME A; ANIMALS; CARNITINE; CATTLE; ELECTRON TRANSPORT COMPLEX III; ENERGY METABOLISM; HUMANS; MITOCHONDRIA; REYE SYNDROME;

EID: 52949084673     PISSN: 00052728     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbabio.2008.05.447     Document Type: Article

References (61)

1. Kashfi K., and Cook G.A. Topology of hepatic mitochondrial carnitine palmitoyltransferase I. Adv. Exp. Med. Biol. 466 (1999) 27-42
2. Ventura F.V., Ijlst L., Ruiter J., Ofman R., Cost C.G., Jakobs C., Duran M., Tavares de Almeida I., Bieber L.L., and Wanders R.J. Carnitine palmitoyltransferase II specificity towards beta-oxidation intermediates-evidence for a reverse carnitine cycle in mitochondria. Eur. J. Biochem. 253 (1998) 614-618
3. Kölker S., Koeller D.M., Okun J.G., and Hoffmann G.F. Pathomechanisms of neurodegeneration in glutaryl-CoA dehydrogenase deficiency. Ann. Neurol. 55 (2004) 7-12
4. Sauer S.W., Okun J.G., Schwab M.A., Crnic L.R., Hoffmann G.F., Goodman S.I., Koeller D.M., and Kolker S. Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A. J. Biol. Chem. 280 (2005) 21830-21836
5. Zinnanti W.J., Lazovic J., Housman C., LaNoue K., O'Callaghan J.P., Simpson I., Woontner M., Goodman S.I., Connor J.R., Jacobs R.E., and Cheng K.C. Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I. J. Clin. Invest. 117 (2007) 3258-3270
6. Schwab M.A., Sauer S.W., Okun J.G., Nijtmans L.G.J., Rodenburg R.J.T., Van den Heuvel L.P., Dröse S., Brandt U., Hoffmann G.F., Ter Laak H., Kölker S., and Smeitink J.A.M. Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins. Biochem. J. 398 (2006) 107-112
7. Okun J.G., Hörster F., Farkas L.M., Feyh P., Hinz A., Sauer S., Hoffmann G.F., Unsicker K., Maytepek E., and Kölker S. Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity. J. Biol. Chem. 277 (2002) 14674-14680
8. Kölker S., Schwab M., Hörster F., Sauer S., Hinz A., Wolf N.I., Mayatepek E., Hoffmann G.F., Smeitink J.A.M., and Okun J.G. Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain. J. Biol. Chem. 278 (2003) 47388-47393
9. Morath M.A., Okun J.G., Müller I.B., Sauer S.W., Hörster F., Hoffmann G.F., and Kölker S. Neurodegeneration and chronic renal failure in methylmalonic aciduria-A pathophysiological approach. J. Inherit. Metab. Dis. S. (2007) epub ahead of print
10. Lai J.C., Liang B.B., Jarvi E.J., Cooper A.J., and Lu D.R. Differential effects of fatty acyl coenzyme A derivatives on citrate synthase and glutamate dehydrogenase. Res. Commun. Chem. Pathol. Pharmacol. 82 (1993) 331-338
11. Kawaguchi A., and Bloch K. Inhibition of glutamate dehydrogenase and malate dehydrogenases by palmitoyl coenzyme A. J. Biol. Chem. 10 (1976) 1406-1412
12. Ventura F.V., Ruiter J.P., Ijlst L., Almeida I.T., and Wanders R.J. Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders. Biochim. Biophys. Acta 1272 (1995) 14-20
13. Ventura F.V., Ruiter J., Ijlst L., de Almeida I.T., and Wanders R.J. Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects. Mol. Genet. Metab. 86 (2005) 344-352
14. Ciapaite J., van Eikenhorst G., and Krab K. Application of modular control analysis to inhibition of the adenine nucleotide translocator by palmitoyl-CoA. Mol. Biol. Rep. 29 (2002) 13-16
15. Moore K.H., Dandurand D.M., and Kiechle F.L. Fasting induced alterations in mitochondrial palmitoyl-CoA metabolism may inhibit adipocyte pyruvate dehydrogenase activity. Int. J. Biochem. 24 (1992) 809-814
16. Kennett F.F., Knauer T.E., Owens K., and Weglicki W.B. Inhibition of myocardial rotenone-insensitive NADH cytochrome c reductase by amphiphilic compounds. Am. J. Physiol. 247 (1984) H889-H894
17. Lichtenstein A.H., Small D.M., and Brecher P. Interaction of oleoyl coenzyme A with phospholipid bilayers. Biochemistry 21 (1982) 2233-2241
18. Wood P.A. Defects in mitochondrial beta-oxidation of fatty acids. Curr. Opin. Lipidol. 10 (1999) 107-112
19. Okun J.G., Lümmen P., and Brandt U. Three classes of inhibitors share a common binding domain in mitochondrial complex I (NADH:ubiquinone oxidoreductase). J. Biol. Chem. 274 (1999) 2625-2630
20. Lowry O.H., Roseborough N.R., and Farr A.J. Protein measurement with the Folin phenol reagent. J. Biol. Chem. 193 (1951) 265-275
21. Helenius A., and Simons K. The binding of detergents to lipophilic and hydrophilic proteins. J. Biol. Chem. 247 (1972) 3656-3661
22. Ziegler D., and Rieske J.S. Preparation and properties of succinate dehydrogenase-coenzyme Q reductase (complex II). Methods Enzymol. 10 (1967) 231-235
23. Brandt U., and Okun J.G. Role of deprotonation events in ubihydroquinone:cytochrome c oxidoreductase from bovine heart and yeast mitochondria. Biochemistry 36 (1997) 11234-11240
24. Sinjorgo K.M., Durak I., Dekker H.L., Edel C.M., Hakvoort T.B., van Gelder B.F., and Muijsers A.O. Bovine cytochrome c oxidases, purified from heart, skeletal muscle, liver and kidney, differ in the small subunits but show the same reaction kinetics with cytochrome c. Biochim. Biophys. Acta 893 (1987) 251-258
25. Percy J.M., Pryde J.G., and Apps D.K. Isolation of ATPase I, the proton pump of chromaffin-granule membranes. Biochem. J. 231 (1985) 557-564
26. Humphries K.M., and Szweda L.I. Selective inactivation of alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase: reaction of lipoic acid with 4-hydroxy-2-nonenal. Biochemistry 37 (1998) 15835-15841
27. Chretien D., Pourrier M., Bourgeron T., Sene M., Rötig A., Munnich A., and Rustin P. An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle. Clin. Chim. Acta 240 (1995) 129-136
28. Ackrell B.A., Kearney E.B., and Singer T.P. Effect of membrane environment on succinate dehydrogenase activity. J. Biol. Chem. 252 (1977) 1582-1588
29. Yu C.A., and Yu L. Structural role of phospholipids in ubiquinol-cytochrome c reductase. Biochemistry 19 (1980) 5715-5720
30. Schägger H., Hagen T., Roth B., Brandt U., Link T.A., and von Jagow G. Phospholipid specificity of bovine heart bc1 complex. Eur. J. Biochem. 190 (1990) 123-130
31. Gomez Jr. D., and Robinson N.C., Phospholipase digestion of bound cardiolipin reversibly inactivates bovine cytochrome bc1. Biochemistry 38 (1999) 9031-9038
32. Kearney E.B., Mayr M., and Singer T.P. Regulatory properties of succinate dehydrogenase: activation by succinyl CoA, pH, and anions. Biochem. Biophys. Res. Commun. 46 (1972) 531-537
33. Gutierrez-Cirlos E.B., Merbitz-Zahradnik T., and Trumpower B.L. Inhibition of the yeast cytochrome bc1 complex by ilicicolin H, a novel inhibitor that acts at the Qn site of the bc1 complex. J. Biol. Chem. 279 (2004) 8708-8714
34. Kamensky Y., Konstantinov A.A., Kunz W.S., and Surkov S. Effects of bc1-site electron transfer inhibitors on the absorption spectra of mitochondrial cytochromes b. FEBS Lett. 181 (1985) 95-99
35. Covian R., Zwicker K., Rotsaert F.A., and Trumpower B.L. Asymmetric and Redox-specific binding of quinone and quinol at center N of the dimeric yeast cytochrome bc1 complex. J. Biol. Chem. 282 (2007) 24198-24208
36. Zhang Z., Huang L., Shulmeister V.M., Chi Y.I., Kim K.K., Hung L.W., Crofts A.R., Berry E.A., and Kim S.H. Iron transfer by domain movement in cytochrome bc1. Nature 392 (1998) 677-684
37. Corkey B.E., Hale D.E., Glennon M.C., Kelley R.I., Coates P.M., Kilpatrick L., and Stanley C.A. Relationship between unusual hepatic acyl coenzyme A profiles and the pathogenesis of Reye syndrome. J. Clin. Invest. 82 (1988) 782-788
38. Roe C., and Coates P. Mitochondrial fatty acid oxidation disorders. In: Scriver C., Beaudet A., Sly W., and Valle D. (Eds). The Metabolic and Molecular Basis of Inherited Disease (1997), McGraw Hill, New York 1501-1534
39. Iafolla A.K., Thompson R.J., and Roe C.R. Medium-chain acyl-coenzyme-A dehydrogenase-deficiency: clinical course in 120 affected children. J. Pediatr. 124 (1994) 409-415
40. Touma E.H., and Charpentier C. Medium chain acyl-CoA dehydrogenase-deficiency. Arch. Dis. Child. 67 (1992) 142-145
41. Derks T.G., Reijngoud D.J., Waterham H.R., Gerver W.J., van den Berg M.P., Sauer P.J., and Smit G.P. The natural history of medium-chain acyl CoA dehydrogenase deficiency in the Netherlands: clinical presentation and outcome. J. Pediatr. 148 (2006) 665-670
42. Stanley C.A., Hale D.E., Coates P.M., Hall C.L., Corkey B.E., Yang W., Kelley R.I., Gonzales E.L., Williamson J.R., and Baker L. Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine level. Pediat. Res. 17 (1983) 877-884
43. Lake B.D., Clayton P.T., Leonard J.V., Bhuiyan A.K.M.J., and Aynsley-Green A. Ultrastructure of liver in inherited disorders of fat oxidation. Lancet I (1987) 382-383
44. Santer R., Schmidt-Sommerfeld E., Leung Y.K., Fischer J.E., and Lebenthal E. Medium-chain acyl CoA dehydrogenase deficiency: electron microscopic differentiation from Reye syndrome. Eur. J. Pediatr. 150 (1990) 111-114
45. Smith E.T., and Gregory J.D. Medium-chain acylcoenzyme-A dehydrogenase deficiency. Am. J. Forensic Med. Pathol. 14 (1993) 313-318
46. Roe C.R., Millington D.S., Maltby D.A., and Kinnebrew P. Recognition of medium-chain acyl-CoA dehydrogenase deficiency in asymptomatic siblings of children of sudden infant death or Reye-like syndrome. J. Pediatr. 108 (1986) 13-18
47. Treem W.R., Witzleben C.A., Piccoli D.A., Stanley C.A., Hale D.E., Coates P.M., and Watkins J.B. Medium-chain and long-chain acyl-CoA dehydrogenase deficiency: clinical and ultrastructural differentiation from Reye syndrome. Hepatology 6 (1986) 1270-1278
48. Wilson G.N., de Chadarévian J.P., Kaplan P., Loehr J.P., Frerman F.E., and Goodman S.I. Glutaric aciduria type II: review of the phenotype and report of an unusual glomerulopathy. Am. J. Med. Genet. 32 (1989) 395-401
49. Loehr J.P., Goodman S.I., and Frerman F.E. Glutaric acidemia type II: heterogeneity of clinical and biochemical phenotypes. Pediatr. Res. 27 (1990) 311-315
50. Reye R.D.K., Morgan G., and Baral J. Encephalopathy and fatty degeneration of the viscera. A disease entity in childhood. Lancet 2 (1963) 749-752
51. Starko K.M., Ray C.G., Dominguez L.B., Stromberg W.L., and Woodall D.F. Reye's syndrome and salicylate use. Pediatrics 66 (1980) 859-864
52. Waldman R.J., Hall W.N., McGee H., and van Amburg G. Apirin as a risk factor in Reye's syndrome. JAMA 247 (1982) 3089-3094
53. Hurwitz E.S., Barrett M.J., Bregman D., Gunn W.J., Schonberger L.B., Fairweather W.R., Drage J.S., LaMontagne J.R., Kaslow R.A., and Burlington D.B. Public Health Service study on Reye's syndrome and medications: report of the pilot phase. N. Engl. J. Med. 313 (1985) 849-857
54. Hurwitz E.S., Barrett M.J., Bregman D., Gunn W.J., Pinsky P., Schonberger L.B., Drage J.S., Kaslow R.A., Burlington D.B., and Quinnan G.V. Public Health Service study on Reye's syndrome and medications: report of the main study. JAMA 257 (1985) 1905-1911
55. Casteels-van Daele M., van Geet C., Wouters C., and Eggermont E. Reye syndrome revisited: a descriptive term covering a group of heterogenous disorders. Eur. J. Pediatr. 159 (2000) 641-648
56. Orlowski J.P., Gillis J., and Kilham H.A. A catch in the Reye. Pediatrics 80 (1987) 638-642
57. Partin J.C., Schubert W.K., and Partin J.S. Mitochondrial ultrastructure in Reye's syndrome (encephalopathy and fatty degeneration of the viscera). N. Engl. J. Med 285 (1971) 1339-1393
58. Ikeda Y., Dabrowski C., and Tanaka K. Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase. J. Biol. Chem. 258 (1983) 1066-1076
59. Ikeda Y., and Tanaka K. Purification and characterization of 2-methyl-branched chain acyl coenzyme A dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria. J. Biol. Chem. 258 (1983) 9477-9487
60. Trauner D.A., Nyhan W.L., and Sweetman L. Jamaican vomiting sickness and Reye's syndrome. Engl. J. Med. 295 (1976) 1481-1482
61. Lieu Y.K., Hsu B.Y., Price W.A., Corkey B.E., and Stanley C.A. Carnitine effects on coenzyme A profiles in rat liver with hypoglycin inhibition of multiple dehydrogenases. Am. J. Physiol. 272 (1997) E359-E366