{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T12:16:25Z","timestamp":1781784985907,"version":"3.54.5"},"publisher-location":"Heidelberg","reference-count":94,"publisher":"Steinkopff","isbn-type":[{"value":"9783662083925","type":"print"},{"value":"9783662083901","type":"electronic"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1987]]},"DOI":"10.1007\/978-3-662-08390-1_8","type":"book-chapter","created":{"date-parts":[[2013,2,2]],"date-time":"2013-02-02T13:31:41Z","timestamp":1359811901000},"page":"63-73","source":"Crossref","is-referenced-by-count":2,"title":["The role of the carnitine system in myocardial fatty acid oxidation: carnitine deficiency, failing mitochondria and cardiomyopathy"],"prefix":"10.1007","author":[{"given":"H. R.","family":"Scholte","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I. E. M.","family":"Luyt-Houwen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. H. M.","family":"Vaandrager-Verduin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","reference":[{"key":"8_CR1","first-page":"265","volume":"29","author":"DS Alberts","year":"1978","unstructured":"Alberts DS, Pen Y-M, Moon TE, Bressler R (1978) Carnitine prevention of adriamycin toxicity in mice. Biomedicine 29: 265\u2013268","journal-title":"Biomedicine"},{"key":"8_CR2","first-page":"327","volume":"62","author":"PG Barth","year":"1983","unstructured":"Barth PG, Scholte HR, Berden JA, van der Klei-van Moorsel JM, Luyt-Houwen IEM, van \u2018t Veer-Korthof ETh, van der Harten JJ, Sobotka-Plojhar MA (1983) An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes. J Neurol Sci 62: 327\u2013355","journal-title":"An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes. J Neurol Sci"},{"key":"8_CR3","first-page":"13","volume-title":"Intravenous DL-carnitine fails to increase the double product during atrial pacing in patients with effort angina","author":"A Biaggini","year":"1983","unstructured":"Biaggini A, Opie L, Rovai D, Mazzei MG, Carpeggiani C, Maseri A. (1983) Intravenous DL-carnitine fails to increase the double product during atrial pacing in patients with effort angina. A double blind randomized study. G Ital Cardiol 13: 291\u2013294"},{"key":"8_CR4","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1016\/S1874-6047(08)60316-9","volume":"16","author":"LL Bieber","year":"1983","unstructured":"Bieber LL, Farrell S (1983) Carnitine acyltransferases. The enzymes 16: 627\u2013644","journal-title":"The enzymes"},{"key":"8_CR5","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1016\/S0140-6736(78)90422-1","volume":"1","author":"T Bohmer","year":"1978","unstructured":"Bohmer T, Bergrem H, Eiklid K (1978) Carnitine deficiency induced during intermittent hemodialysis for renal failure. Lancet 1: 126\u2013128","journal-title":"Lancet"},{"key":"8_CR6","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1016\/0006-2944(78)90037-6","volume":"19","author":"H Bookelman","year":"1978","unstructured":"Bookelman H, Trijbels JMF, Sengers RCA, Janssen AJM (1978) Measurement of cytochromes in human skeletal muscle mitochondria isolated from fresh and frozen stored muscle specimens. Biochem Med 19: 366\u2013373","journal-title":"Biochem Med"},{"key":"8_CR7","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1146\/annurev.nu.03.070183.001313","volume":"3","author":"PR Borum","year":"1983","unstructured":"Borum PR (1983) Carniture. Ann Rev Nutr 3: 233\u2013259","journal-title":"Carniture. Ann Rev Nutr"},{"key":"8_CR8","doi-asserted-by":"crossref","first-page":"1320","DOI":"10.1152\/physrev.1983.63.4.1420","volume":"63","author":"J Bremer","year":"1983","unstructured":"Bremer J (1983) Carnitine-metabolism and functions. Physiol Rev 63: 1320\u20131479","journal-title":"Physiol Rev"},{"key":"8_CR9","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/S0167-7306(08)60123-4","volume-title":"Fatty acid metabolism and its regulation","author":"J Bremer","year":"1984","unstructured":"Bremer J, Osmondsen H (1984) Fatty acid oxidation and its regulation. In: Numa S (ed) Fatty acid metabolism and its regulation, Elsevier, Amsterdam, pp 113\u2013154"},{"key":"8_CR10","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/0304-4165(65)90217-5","volume":"104","author":"R Bressler","year":"1965","unstructured":"Bressler R, Wittels B (1965) The effect of diphteria toxin on carnitine metabolism in the heart. Biochim Biophys Acta 104: 39\u201345","journal-title":"Biochim Biophys Acta"},{"key":"8_CR11","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1042\/bj2020077","volume":"202","author":"RW Brownsey","year":"1982","unstructured":"Brownsey RW, Denton RM (1982) Evidence that insulin activates fatcell acetyl-CoA carboxylase by increased phosphorylation at a specific site. Biochem J 202: 77\u201386","journal-title":"Biochem J"},{"key":"8_CR12","first-page":"616","volume":"77","author":"DR Chaloner","year":"1971","unstructured":"Chaloner DR, Mandelbaum I, Elliott W (1971) Protective effect of L-carnitine in experimental intoxication with diphteria toxin. J Lab Clin Med 77: 616\u2013628","journal-title":"J Lab Clin Med"},{"key":"8_CR13","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/0003-9861(82)90450-7","volume":"213","author":"D Davidson","year":"1982","unstructured":"Davidson D, Schultz H (1982) Separation, properties and regulation of acyl-CoA dehydrogenase from bovine heart and liver. Arch Biochem Biophys 213: 155\u2013162","journal-title":"Arch Biochem Biophys"},{"key":"8_CR14","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1016\/0005-2760(86)90053-6","volume":"876","author":"CE Douglas","year":"1986","unstructured":"Douglas CE, Chan AC, Choy PC (1986) Vitamin E inhibits platelet phospholipase A2. Biochim Biophys Acta 876: 639\u2013645","journal-title":"E inhibits platelet phospholipase A2. Biochim Biophys Acta"},{"key":"8_CR15","first-page":"1663","volume-title":"Engel Ag","author":"AG Engel","year":"1986","unstructured":"Engel AG (1986) Carnitine deficiency syndromes and lipid storage myopathies. In: Engel AG, Banker BQ (eds) Myology; McGraw-Hill, New York, pp 1663\u20131696"},{"key":"8_CR16","first-page":"31","volume-title":"Primary systematic carnitine deficiency","author":"AG Engel","year":"1981","unstructured":"Engel AG, Rebouche CJ, Wilson DM, Glasgow AM, Romshe CA, Cruse RP (1981) Primary systematic carnitine deficiency. II Renal handling of carnitine. Neurology 31: 819\u2013825"},{"key":"8_CR17","doi-asserted-by":"publisher","first-page":"193","DOI":"10.3109\/00365518509160995","volume":"45","author":"B Fagher","year":"1985","unstructured":"Fagher B, Cederblad G, Monti, M. Olsson L, Rasmussen B, Thyssell H (1985) Carnitine and left ventricular function in haemodialysis patients. Scand J Clin Lab Invest 45: 193\u2013198","journal-title":"Scand J Clin Lab Invest"},{"key":"8_CR18","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1016\/0167-5273(84)90149-9","volume":"5","author":"R Ferrari","year":"1984","unstructured":"Ferrari R, Cucchini F, Visiolo 0 (1984) The metabolic effects of L-carnitine in angina pectoris. Int J Cardiol 5: 213\u2013216","journal-title":"The metabolic effects of L-carnitine in angina pectoris. Int J Cardiol"},{"key":"8_CR19","doi-asserted-by":"publisher","first-page":"4513","DOI":"10.1073\/pnas.82.13.4513","volume":"82","author":"K Folkers","year":"1985","unstructured":"Folkers K, Wolaniuk J, Simonsen, R, Morishita M, Vadhanavikit S (1985) Biochemical rationale and the cardiac response of patients with muscle disease to therapy with coenzyme Q10. Proc Natl Acad Sci USA 82: 4513\u20134516","journal-title":"Proc Natl Acad Sci Usa"},{"key":"8_CR20","doi-asserted-by":"publisher","first-page":"1209","DOI":"10.1016\/0002-9149(78)90877-9","volume":"41","author":"JD Folts","year":"1978","unstructured":"Folts JD, Shug AL, Koke JR, Bittar N (1978) Protection of the ischemic dog myocardium with carnitine. Am J Cardiol 41: 1209\u20131214","journal-title":"Am J Cardiol"},{"key":"8_CR21","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1203\/00006450-198305000-00003","volume":"17","author":"AM Glasgow","year":"1983","unstructured":"Glasgow AM, Engel AG, Bier DM, Perry LW, Dicki M, Todaro J, Brown BI, Utter MF (1983) Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain carnitine excess responsive to medium chain triglyceride diet. Pediat Res 17: 319\u2013326","journal-title":"Pediat Res"},{"key":"8_CR22","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/B978-0-12-024914-5.50009-7","volume":"14","author":"PE Groot","year":"1976","unstructured":"Groot PHE, Scholte HR, H\u00fclsman WC (1976) Fatty acid activation: specificity, localization and function. Adv Lipid Res 14: 75\u2013126","journal-title":"Adv Lipid Res"},{"key":"8_CR23","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1203\/00006450-198507000-00006","volume":"19","author":"DE Hale","year":"1985","unstructured":"Hale DE, Batshaw ML, Coates PM, Frerman FE, Goodman SI, Sing I, Stanley CA (1985) Long-chain acyl-CoA dehydrogenase deficiency: an inherited cause of non-ketotic hypoglycemia. Pediat Res 19: 666\u2013671","journal-title":"Pediat Res"},{"key":"8_CR24","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1016\/0014-5793(85)80385-9","volume":"188","author":"Y Harano","year":"1985","unstructured":"Harano Y, Kashiwagi A, Kojima H, Suzuki M, Hashimoto T, Shigeta Y (1985) Phosphorylation of carnitine palmitoyltransferase and activation by glucagon in isolated rat hepatocytes. FEBS Lett 188: 267\u2013272","journal-title":"Febs Lett"},{"key":"8_CR25","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1212\/WNL.28.2.147","volume":"28","author":"ZH Hart","year":"1978","unstructured":"Hart ZH, Chang C-H, DiMauro S, Farooki Q, Ayyar R (1978) Muscle carnitine deficiency and fatal cardiomyopathy. Neurology 28: 147\u2013151","journal-title":"Neurology"},{"key":"8_CR26","first-page":"56","volume":"1","author":"DJ Hearse","year":"1980","unstructured":"Hearse DJ, Shattoch MJ, Manning AS (1980) Carnitine in cardioplegic solutions: possible toxicity during global ischemia. J Cell Mol Cardiol 12, suppl 1: 56","journal-title":"J Cell Mol Cardiol 12, suppl"},{"key":"8_CR27","doi-asserted-by":"publisher","first-page":"754","DOI":"10.1016\/S0022-3476(81)80404-0","volume":"99","author":"CG Henry","year":"1981","unstructured":"Henry CG, Strauss AW, Keating JP, Hillman RE (1981) Congestive cardiomyopathy associated with 13-ketothiolase deficiency. J Pediatr 99: 754\u2013757","journal-title":"J Pediatr"},{"key":"8_CR28","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1042\/bj2260139","volume":"226","author":"R Holland","year":"1985","unstructured":"Holland R, Hardie DG, Clegg RA, Zammit VA (1985) Evidence that glucagon-mediated inhibition of acetyl-CoA carboxylase in isolated hepatocytes involves increased phosphorylation of the enzyme by cyclic AMP-dependent protein kinase. Biochem J 226: 139\u2013145","journal-title":"Biochem J"},{"key":"8_CR29","doi-asserted-by":"publisher","first-page":"798","DOI":"10.1203\/00006450-198408000-00039","volume":"18","author":"JM Huijmans","year":"1984","unstructured":"Huijmans JGM, Schulte HR, Blom W, Luyt-Houwen IEM, Przyrembel H (1984) Enzymatic evidence for a medium-chain acyl-CoA dehydrogenase deficiency in muscle of a patient with hypoketotic hypoglycemic dicarboxylic aciduria. Pediat Res 18: 798","journal-title":"Pediat Res"},{"key":"8_CR30","doi-asserted-by":"publisher","first-page":"398","DOI":"10.1016\/0005-2760(66)90081-6","volume":"125","author":"WC H\u00fclsman","year":"1966","unstructured":"H\u00fclsman WC (1966) On the synthesis of malonyl-coenzyme A in rabbit heart sarcosomes. Biochim Biophys Acta 125: 398\u2013400","journal-title":"Biochim Biophys Acta"},{"key":"8_CR31","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0303-7207(78)90096-5","volume":"12","author":"WC H\u00fclsman","year":"1978","unstructured":"H\u00fclsman WC (1978) Abnormal stress reactions after feeding diets rich in (very) long-chain fatty acids: high levels of corticosterone and testosterone. Mol Cell Endocrinol 12: 1\u20138","journal-title":"Mol Cell Endocrinol"},{"key":"8_CR32","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/0005-2760(82)90164-3","volume":"713","author":"WC H\u00fclsman","year":"1982","unstructured":"H\u00fclsman WC, Stam H, Maccari F (1982) The effect of excess (acyl)-carnitine on lipid metabolism in rat heart. Biochim Biophys Acta 713: 39\u201345","journal-title":"Biochim Biophys Acta"},{"key":"8_CR33","first-page":"213","volume-title":"Mitochondria and muscular diseases","author":"FI Jennekens","year":"1981","unstructured":"Jennekens FGI, Scholte HR, Stinis JT, Luyt-Houwen IEM (1981) Carnitine palmitoyltransferase deficiency, variations in clinical expression, differences between CPT I and II and mode of inheritance. In: Busch HFM, Jennekens FGI, Scholte HR (eds) Mitochondria and muscular diseases, Mefar, Beetsterzwaag (The Netherlands), pp 213\u2013218"},{"key":"8_CR34","first-page":"753","volume":"30","author":"P Kolsolcharoen","year":"1981","unstructured":"Kolsolcharoen P, Nappi J, Peduzzi P, Shug A, Patel A, Filipek T, Thomsen JH (1981) Improved exercise tolerance after administration of carnitine. Curr Ther Res. 30: 753\u2013764","journal-title":"Curr Ther Res"},{"key":"8_CR35","doi-asserted-by":"publisher","first-page":"1391","DOI":"10.1253\/jcj.47.1391","volume":"47","author":"Y Kudoh","year":"1983","unstructured":"Kudoh Y, Shoji T, Oimatsu H, Yoshida S, Kikuchi K, Iimura O (1983) The role of L-carnitine in the pathogenesis of cardiomegaly in patients with chronic hemodialysis. Jpn Circ J 47: 1391\u20131397","journal-title":"Jpn Circ J"},{"key":"8_CR36","doi-asserted-by":"publisher","first-page":"4220","DOI":"10.1073\/pnas.82.12.4240","volume":"82","author":"PH Langsjoen","year":"1985","unstructured":"Langsjoen PH, Vadhanavikit S, Folkers K (1985) Response of patients in classes III and IV of cardiomyopathy to therapy in a blind and crossover trial with coenzyme Q1o. Proc Natl Acad Sci USA 82: 4220\u20134244","journal-title":"Proc Natl Acad Sci Usa"},{"key":"8_CR37","doi-asserted-by":"publisher","first-page":"331","DOI":"10.1016\/0005-2728(77)90219-5","volume":"460","author":"GM Lauquin","year":"1977","unstructured":"Lauquin GJM, Villiers C, Mechejda JW, Hrniewiecka LV, Vignais PV (1977) Adenine nucleotide transport in sonic submitochondrial particles. kinetc properties and binding of specific inhibitors. Biochim Biophys Acta 460: 331\u2013345","journal-title":"Biochim Biophys Acta"},{"key":"8_CR38","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1161\/01.RES.48.6.859","volume":"48","author":"AJ Liedtke","year":"1981","unstructured":"Liedtke AJ, Nellis SH, Whitesell LF (1981) Effects of carnitine isomers on fatty acid metabolism in ischemic swine hearts. Circ Res 48: 859\u2013866","journal-title":"Circ Res"},{"key":"8_CR39","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1212\/WNL.32.6.663","volume":"32","author":"CS Long","year":"1982","unstructured":"Long CS, Haller RG, Foster DW, McGarry JD (1982) Kinetics of carnitine-dependent fatty acid oxidation: implications for human carnitine deficiency. Neurology 32: 663\u2013666","journal-title":"Neurology"},{"key":"8_CR40","first-page":"65","volume":"35","author":"F Maccari","year":"1981","unstructured":"Maccari F, Ramacci MT (1981) Antagonism of doxorubicin cardiotoxicity by carnitine is specific of the L-diasteroisomer. Biomedicine 35: 65\u201367","journal-title":"Biomedicine"},{"key":"8_CR41","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1042\/bj2140021","volume":"214","author":"JD Mcgarry","year":"1983","unstructured":"McGarry JD, Mills SE, Long CS, Foster DW (1983) Observations on the affinity for carnitine, and malonyl-CoA sensitivity, of carnitine palmitoyltransferase I in animal and human tissues. Biochem J 214: 21\u201328","journal-title":"Biochem J"},{"key":"8_CR42","doi-asserted-by":"publisher","first-page":"6","DOI":"10.1055\/s-2008-1052536","volume":"16","author":"T Matsuishi","year":"1985","unstructured":"Matsuishi T, Hirata K, Terasawa K, Kato H, Yoshino M, Ohtaki E, Hirose F (1985) Successful carnitine treatment in two siblings having lipid storage myopathy with hypertrophie cardiomyopathy. Neuropediat 16: 6\u201312","journal-title":"Neuropediat"},{"key":"8_CR43","first-page":"641","volume-title":"The effect of diphteria toxin on the cellular uptake and efflux of carnitine","author":"P Molstad","year":"1981","unstructured":"Molstad P, Bohmer T (1981) The effect of diphteria toxin on the cellular uptake and efflux of carnitine. Evidence for a protective effect of prednisolone. Biochim Biophys Acta 641: 71\u201378"},{"key":"8_CR44","unstructured":"Morand P, Despert F, Carrier HN, Saudubray JM, Fardeau M, Romieux B, Fauchier C, Combe P. Myopathie avec cardiomyopathie s\u00e9v\u00e8re par d\u00e9ficit g\u00e9neralis\u00e9 en carnitine. Evolution favorable sous un traitement par chlorohydrate de carnitine. Arch Mal Coeur 5: 536\u2013544"},{"key":"8_CR45","doi-asserted-by":"publisher","first-page":"685","DOI":"10.1172\/JCI111484","volume":"47","author":"RW Moreadith","year":"1984","unstructured":"Moreadith RW, Batshaw ML, Ohnishi T, Kerr D, Knox B, Jackson D, Hruban R, Olson J, Reynafarje B, Lehninger AL (1984) Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamideadenine-dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis. J Clin Invest 47: 685\u2013697","journal-title":"J Clin Invest"},{"key":"8_CR46","first-page":"15","volume":"9","author":"JA Morgan-Hughes","year":"1986","unstructured":"Morgan-Hughes JA (1986) Mitochondrial diseases. TINS 9: 15\u201319","journal-title":"Tins"},{"key":"8_CR47","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1042\/bst0130648","volume":"13","author":"JA Morgan-Hughes","year":"1985","unstructured":"Morgan-Hughes JA, Hayes DJ, Cooper CM, Clark JB (1985) Mitochondrial myopathies: deficiencies localized to complex I and complex III of the mitochondrial respiratory chain. Biochem Soc Trans 13: 648\u2013650","journal-title":"Biochem Soc Trans"},{"key":"8_CR48","first-page":"38","volume-title":"Multiple acyl-CoA dehydrogenase deficiency in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy","author":"A Niederwieser","year":"1983","unstructured":"Niederwieser A, Steinmann B, Exner U, Neuheiser F, Redweik U, Wang M, Rampini S, Wendel U (1983) Multiple acyl-CoA dehydrogenase deficiency in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy. Detection of N-isovalerylglutamic acid and its monamide. Helv Paediatr Acta 38: 9\u201326"},{"key":"8_CR49","first-page":"225","volume-title":"Ischemic myocardium and antianginal drugs","author":"JR Neely","year":"1979","unstructured":"Neely JR, Garber D, McDonough K, Idell-Wenger J (1979) Relationship between ventricular function and intermediates of fatty acid metabolism during myocardial ischemia: effects of carnitine. In: Win-bury MM, Abiko Y (eds) Ischemic myocardium and antianginal drugs, Raven Press, New York, pp 225\u2013234"},{"key":"8_CR50","first-page":"529","volume":"72","author":"J Norman","year":"1979","unstructured":"Norman J, Carrier H, Berthillier G, Bozio A, Jocteur-Monrozier D, Andr\u00e9 M, Joffre B (1979) Myocardiopathie primitive de l\u2019enfant avec surcharge lipidique des fibres myocardiques et musculaires et mise en \u00e9vidence d\u2019un d\u00e9ficit en enzyme palmityl-carnitine transf\u00e9rase. Arch Mal Coeur 72: 529\u2013535","journal-title":"Arch Mal Coeur"},{"key":"8_CR51","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1212\/WNL.36.1.45","volume":"36","author":"S Ogasahara","year":"1986","unstructured":"Ogasahara S, Nishikawa Y, Yorifuji S, Soga F, Nakamura Y, Takahashi M, Hashimoto S, Kono N, Tarui S (1986) Treatment of Kearns-Sayre syndrome with coenzyme Q1o. Neurology 36: 45\u201353","journal-title":"Neurology"},{"key":"8_CR52","doi-asserted-by":"publisher","first-page":"372","DOI":"10.1212\/WNL.35.3.372","volume":"35","author":"S Ogashara","year":"1985","unstructured":"Ogashara S, Yorifuji S, Nishikawa Y, Takahashi M, Wada K, Hazama T, Nakamura Y, Hashimoto S, Kono N, Tarui S (1985) Improvement of abnormal pyruvate metabolism and cardiac conduction defect with coenzyme Qio in Kearns-Sayre syndrome. Neurology 35: 372\u2013377","journal-title":"Neurology"},{"key":"8_CR53","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1016\/0002-8703(79)90440-X","volume":"97","author":"LH Opie","year":"1979","unstructured":"Opie LH (1979) Role of carnitine in fatty acid metabolism of normal and ischemic myocardium. Am J Heart 97: 375\u2013388","journal-title":"Am J Heart"},{"key":"8_CR54","first-page":"233","volume":"877","author":"SV Pande","year":"1986","unstructured":"Pande SV, Murthy MSR, No\u00ebl H (1986) Differential effects of phosphatidylcholine and cardiolipin on carnitine palmitoyltransferase activity. Biochim Biophys Acta 877: 233\u2013240","journal-title":"Biochim Biophys Acta"},{"key":"8_CR55","first-page":"1023","volume":"18","author":"A Papadimitriou","year":"1984","unstructured":"Papadimitriou A, Neustein HB, DiMauro S, Stanton R, Bresolin N (1984) Histocytoid cardiomyopathy of infancy: deficiency of reducible cytochrome b in heart mitochondria. Pediat Res 18: 1023\u20131028","journal-title":"Pediat Res"},{"key":"8_CR56","doi-asserted-by":"publisher","first-page":"976","DOI":"10.1016\/S0022-3476(81)80609-9","volume":"98","author":"D Penn","year":"1981","unstructured":"Penn D, Schmidt-Sommerfeld E, Pascu F (1981) Decreased tissue carnitine concentrations in newborn infants receiving total parenteral nutrition. J Pediat 98: 976\u2013978","journal-title":"J Pediat"},{"key":"8_CR57","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1203\/00006450-198409000-00001","volume":"18","author":"AM Ramos","year":"1984","unstructured":"Ramos ACMF, Elias PRP, Barrucand L, Figueria da Silva JA (1984) The protective effect of carnitine in human diphteric myocarditis. Pediat Res 18: 815\u2013819","journal-title":"Pediat Res"},{"key":"8_CR58","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1016\/0304-4165(80)90133-6","volume":"630","author":"CJ Rebouche","year":"1980","unstructured":"Rebouche CJ, Engel AG (1980) Tissue distribution of carnitine biosynthetic enzymes in man. Biochim Biophys Acta 630: 22\u201329","journal-title":"Biochim Biophys Acta"},{"key":"8_CR59","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1007\/BF01735930","volume":"60","author":"V Regitz","year":"1982","unstructured":"Regitz V, Hodach RJ, Shug AL (1982) Carnitin-Mangel: eine behandelbare Ursache kindlicher Kardiomyopathien. Klin Wschr 60: 393\u2013400","journal-title":"Klin Wschr"},{"key":"8_CR60","doi-asserted-by":"publisher","first-page":"201","DOI":"10.1007\/BF00313387","volume":"227","author":"M Rimoldi","year":"1982","unstructured":"Rimoldi M, Bottacchi E, Rossi L, Cornelio F, Uziel G, DiDonato S (1982) Cytochrome-C-oxidase deficiency in muscles of a floppy infant without mitochondrial myopathy. J Neurol 227: 201\u2013207","journal-title":"J Neurol"},{"key":"8_CR61","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1172\/JCI108824","volume":"60","author":"D Rudman","year":"1977","unstructured":"Rudman D, Sewell CW, Ansley JD (1977) Deficiency of carnitine in cachectic cirrhotic patients. J Clin Invest 60: 716\u2013723","journal-title":"J Clin Invest"},{"key":"8_CR62","first-page":"66","volume-title":"Disruption of the purine nucleotide cycle","author":"RL Sabina","year":"1980","unstructured":"Sabina RL, Swain JL, Patten BM, Ashizawa T, O\u2019Brien WE, Holmes EW (1980) Disruption of the purine nucleotide cycle. A potential explanation for muscle disfunction in myoadenylate deaminase deficiency. J Clin Invest 66: 1419\u20131423"},{"key":"8_CR63","first-page":"1371","volume":"75","author":"A Sacrez","year":"1982","unstructured":"Sacrez A, Porte A, Hindelang C, Bieth R, M\u00e9rian B (1982) Myocardiopathie ayes surcharge lipidique et d\u00e9ficit en palmityl carnitine transf\u00e9rase leucocytaire. Arch Mal Coeur 75: 1371\u20131379","journal-title":"Arch Mal Coeur"},{"key":"8_CR64","doi-asserted-by":"publisher","first-page":"660","DOI":"10.1203\/00006450-197805000-00009","volume":"12","author":"E Schmidt-Sommerfeld","year":"1978","unstructured":"Schmidt-Sommerfeld E, Novak K, Penn D, Wieser PB, Buch M, Hahn P (1978) Carnitine and development of newborn adipose tissue. Pediat Res 12: 660\u2013664","journal-title":"Pediat Res"},{"key":"8_CR65","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1016\/0005-2744(69)90140-5","volume":"178","author":"HR Scholte","year":"1969","unstructured":"Scholte HR (1969) The intracellular and intramitochondrial distribution of malonyl-CoA decarboxylase and propionyl-CoA carboxylase in rat liver. Biochim Biophys Acta 178: 137\u2013144","journal-title":"Biochim Biophys Acta"},{"key":"8_CR66","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1016\/0005-2744(73)90043-0","volume":"309","author":"HR Scholte","year":"1973","unstructured":"Scholte HR (1973) Liver malonyl-CoA decarboxylase. Biochim Biophys Acta 309: 457\u2013465","journal-title":"Biochim Biophys Acta"},{"key":"8_CR67","first-page":"129","volume":"14","author":"HR Scholte","year":"1983","unstructured":"Scholte HR (1983) Carnitine in health and disease. Neuropediatrics 14: 129","journal-title":"Neuropediatrics"},{"key":"8_CR68","first-page":"215","volume-title":"Mitochondrial pathology in muscle diseases","author":"HR Scholte","year":"1983","unstructured":"Scholte HR, Busch HFM, Barth PG, Beekman RP, Berden JA, Duran M, Luyt-Houwen IEM, Przyrembel H, Roth B, De Vries S (1983) Carnitine deficiency and mitochondrial respiratory chain blockade. In: Scarlato G, Cerri C (eds) Mitochondrial pathology in muscle diseases. Piccin, Padua, pp 215\u2013228"},{"key":"8_CR69","doi-asserted-by":"publisher","first-page":"169","DOI":"10.1007\/BF02263644","volume":"4","author":"HR Scholte","year":"1981","unstructured":"Scholte HR, Busch HFM, Luyt-Houwen IEM (1981) Familial AMP deaminase deficiency with skeletal muscle type I atrophy and fatal cardiomyopathy. J Inher Metab Dis 4: 169\u2013170","journal-title":"J Inher Metab Dis"},{"key":"8_CR70","doi-asserted-by":"crossref","unstructured":"Scholte HR, Busch HFM, Luyt-Houwen IEM, Vaandrager-Verduin MHM, Przyrembel H, Arts WFM (1987) Defects in oxidative phosphorylation. Biochemical investigations in skeletal muscle and expression of the lesion in other cells. J Inher Metab Dis, in press","DOI":"10.1007\/BF01812849"},{"key":"8_CR71","unstructured":"Scholte HR, De Jonge PC (1986) Metabolism, function and transport of carnitine in health and disease. In: Gitzelmann R et al. (eds) Carnitin in der Medizin, Schattauer Verlag, Stuttgart, in press"},{"key":"8_CR72","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1042\/bst0130643","volume":"13","author":"HR Scholte","year":"1985","unstructured":"Scholte HR, H\u00fclsmann WC, Luyt-Houwen IEM, Stinis JT, Jennekens FGI (1985a) Carnitine palmitoyltransferase deficiencies. Biochem Soc Trans 13: 643\u2013645","journal-title":"Biochem Soc Trans"},{"key":"8_CR73","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/0022-510X(79)90007-8","volume":"40","author":"HR Scholte","year":"1979","unstructured":"Scholte HR, Jennekens FGI, Bouvy JJBJ (1979a) Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leucocytes. J Neurol Sci 40: 39\u201351","journal-title":"J Neurol Sci"},{"issue":"1808","key":"8_CR74","first-page":"35","volume":"13961397","author":"HR Scholte","year":"1985","unstructured":"Scholte HR, Luyt-Houwen IEM, Busch HFM, Jennekens FGI (1985) Muscle mitochondria from patients with Duchenne muscular dystrophy have a normal beta oxidation, but an impaired oxidative phosphorylation. Neurology 35: 1396\u20131397, 1808","journal-title":"Neurology"},{"key":"8_CR75","first-page":"198","volume-title":"The source of malonyl-CoA in rat heart","author":"HR Scholte","year":"1986","unstructured":"Scholte HR, Luyt-Houwen IEM, Dubelaar M-L, H\u00fclsmann WC (1986) The source of malonyl-CoA in rat heart. In calcium paradox releases acetyl-CoA carboxylase and not propionyl-CoA carboxylase. FEBS Lett 198: 47\u201350"},{"key":"8_CR76","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1016\/S0022-2828(85)80029-8","volume":"17","author":"HP Schultheiss","year":"1985","unstructured":"Schultheiss HP, Bolte HD (1985) Immunological analysis of autoantibodies against the adenine nucleotide translocator in dilated cardiomyopathy. J Mol Cell Cardiol 17: 603\u2013617","journal-title":"J Mol Cell Cardiol"},{"key":"8_CR77","doi-asserted-by":"crossref","unstructured":"Schultheiss H-P, Schultze K, Klingenberg M (1986) The ADP\/ATP carrier as a mitochondria] auto-antigen: facts and perspectives. Abstracts of the conference on biological membrane pathology, Como, Ann NY Acad Sci p5 (in press)","DOI":"10.1111\/j.1749-6632.1986.tb54393.x"},{"key":"8_CR78","doi-asserted-by":"publisher","first-page":"178","DOI":"10.1007\/BF00443221","volume":"141","author":"RA Sengers","year":"1984","unstructured":"Sengers RCA, Trijbels JMF, Bakkeren JAJM, Ruitenbeek W, Fischer JC, Janssen AJM, Stadhouders AM, ter Laak HJ (1984) Deficiency of cytochromes b and aa3 in muscle from a floppy infant with cytochrome oxidase deficiency. Eur J Pediatr 141: 178\u2013180","journal-title":"Eur J Pediatr"},{"key":"8_CR79","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/0003-9861(78)90003-6","volume":"187","author":"AL Shug","year":"1978","unstructured":"Shug AL, Thomson JD, Folts JD, Bittar N, Klein MI, Koke JR, Huth PJ (1978) Changes in tissue levels of carnitine and other metabolites during myocardial ischemia and anoxia. Arch Biochem Biophys 187: 25\u201333","journal-title":"Arch Biochem Biophys"},{"key":"8_CR80","first-page":"804","volume":"14","author":"N Siliprandi","year":"1984","unstructured":"Siliprandi N, di Lisa F, Toninello A (1984) Biochemical derrangements in ischemic myocardium: the role of carnitine. G Ital Cardiol 14: 804\u2013808","journal-title":"G Ital Cardiol"},{"key":"8_CR81","doi-asserted-by":"crossref","unstructured":"Suzuki Y, Masamura Y, Kobayashi A, Kamizaki N, Harida Y, Osawa M (1982) Myocardial carnitine deficiency in chronic heart failure. Lancet is 116","DOI":"10.1016\/S0140-6736(82)90263-X"},{"key":"8_CR82","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0014-5793(83)80330-5","volume":"151","author":"JM Tager","year":"1983","unstructured":"Tager JM, Wanders RJA, Groen AK, Kunz W, Bohnensack R, Mister U, Letko G, B\u00f6hme G, Duszynski J, Wojtczak L (1983) Control of mitochondria] respiration. FEBS Lett 151: 1\u20139","journal-title":"Febs Lett"},{"key":"8_CR83","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1016\/S0002-9149(79)80019-3","volume":"43","author":"JH Thomsen","year":"1979","unstructured":"Thomsen JH, Shug AL Yap VU, Patel AK, Karras TJ, DeFelice SL (1979) Improved pacing tolerance of the ischemic human myocardium after administration of carnitine. Am J Cardiol 43: 300\u2013306","journal-title":"Am J Cardiol"},{"key":"8_CR84","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1056\/NEJM198108133050707","volume":"305","author":"ME Tripp","year":"1981","unstructured":"Tripp ME, Katcher ML, Peters HA, Gilbert EF, Arya S, Hodach RI, Shug AL (1981) Systemic carnitine deficiency presenting as familial fibroelastosis. New Engl J Med 305: 385\u2013390","journal-title":"New Engl J Med"},{"key":"8_CR85","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1016\/0006-2944(84)90073-5","volume":"32","author":"ME Tripp","year":"1984","unstructured":"Tripp ME, Shug AL (1984) Plasma carnitine concentrations in cardiomyopathy patients. Biochem Med 32: 199\u2013206","journal-title":"Biochem Med"},{"key":"8_CR86","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1093\/ajcn\/38.4.532","volume":"38","author":"GM Vacha","year":"1983","unstructured":"Vacha GM, Giorcelli G, Siliprandi N, Corsi M (1983) Favorable effects of L-Carnitine treatment on hypertriglyceridemia in hemodialysis patients: decisive role of low levels of high-carnitine lipoprotein-cholesterol. Am J Clin Nutr 38: 532\u2013540","journal-title":"Am J Clin Nutr"},{"key":"8_CR87","first-page":"256","volume":"20","author":"VM Hinsbergh","year":"1978","unstructured":"Van Hinsbergh VWM, Veerkamp JH, van Moerkerk HThB (1978) An accurate assay of long-chain fatty acid oxidation by human muscle. Biochem Med 20: 256\u2013266","journal-title":"An accurate assay of long-chain fatty acid oxidation by human muscle. Biochem Med"},{"key":"8_CR88","first-page":"190","volume-title":"Palmitate oxidation by rat skeletal muscle mitochondria","author":"VM Hinsbergh","year":"1978","unstructured":"Van Hinsbergh VWM, Veerkamp JH, van Moerkerk HThB (1978) Palmitate oxidation by rat skeletal muscle mitochondria. Comparison of polarographic and radiochemical experiments. Arch Biochem Biophys 190: 762\u2013771"},{"key":"8_CR89","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1016\/0885-4505(86)90080-0","volume":"35","author":"JH Veerkamp","year":"1986","unstructured":"Veerkamp JH, van Moerkerk HTB, Glatz JFC, Zuurveld JGEM, Jacobs AEM, Wagenmakers AJM (1986) 14CO2 production is no adequate measure of [14C] fatty acid oxidation. Biochem Med Metab Biol 35: 248\u2013259","journal-title":"Biochem Med Metab Biol"},{"key":"8_CR90","first-page":"431","volume":"18","author":"JA Vick","year":"1975","unstructured":"Vick JA, De Felice S, Hassett CC (1975) Prevention of adriamycin induced cardiac arrhithmias with carnitine. Physiologist 18: 431","journal-title":"Physiologist"},{"key":"8_CR91","doi-asserted-by":"publisher","first-page":"700","DOI":"10.1016\/S0022-3476(82)80294-1","volume":"101","author":"LJ Waber","year":"1982","unstructured":"Waber LJ, Valle D, Neill C, DiMauro S, Shug A (1982) Carnitine deficiency presenting as familial cardiomyopathy: a treatable defect in carnitine transport. J Pediat 101: 700\u2013705","journal-title":"J Pediat"},{"key":"8_CR92","first-page":"93","volume-title":"Systemic carnitine deficiency","author":"AJ Ware","year":"1978","unstructured":"Ware AJ, Burton WC, McGarry JD, Markes JF, Weinberg AG (1978) Systemic carnitine deficiency. Report of a fatal case with multisystem manifestations. J Pediat 93: 959\u2013964"},{"key":"8_CR93","doi-asserted-by":"crossref","first-page":"4305","DOI":"10.1016\/S0021-9258(17)34720-8","volume":"253","author":"JT Whitmer","year":"1978","unstructured":"Whitmer JT, Idell-Wenger JA, Rovetto MJ, Neely JE (1978) Control of fatty acid metabolism in ischemic and hypoxic hearts. J Biol Chem 253: 4305\u20134309","journal-title":"J Biol Chem"},{"key":"8_CR94","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1016\/0005-2760(70)90032-9","volume":"210","author":"EM Wit-Peeters","year":"1970","unstructured":"Wit-Peeters EM, Scholte HR, Elenbaas HL (1970) Fatty acid synthesis in heart. Biochim Biophys Acta 210: 360\u2013370","journal-title":"Biochim Biophys Acta"}],"container-title":["Lipid metabolism in the normoxic and ischaemic heart"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-3-662-08390-1_8","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,7]],"date-time":"2022-02-07T08:26:26Z","timestamp":1644222386000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-3-662-08390-1_8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1987]]},"ISBN":["9783662083925","9783662083901"],"references-count":94,"URL":"https:\/\/doi.org\/10.1007\/978-3-662-08390-1_8","relation":{},"subject":[],"published":{"date-parts":[[1987]]}}}