Abstract
This chapter will review the data describing the mitochondrial enzyme, carnitine palmitoyltransferase (hexadecanoyl-CoA:carnitine O-hexadecanoyltransferase, EC 2.3.1.23), and its role in the transport and metabolism of long-chain fatty acids.
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References
Aas, M., and Daae, L. N. W., 1971, Fatty acid activation and acyl transfer in organs from rats in different nutritional states, Biochim. Biophys. Acta 239:208–216.
Augenfeld, J., and Fritz, I. B., 1970, Carnitine palmitoyltransferase activity and fatty acid oxidation by livers from fetal and neonatal rats, Can. J. Biochem. 48:288–294.
Barden, R. E., and Cleland, W. W., 1969, Alteration of the concentrations of dilute palmitoyl-CoA solutions by surface adsorption, Biochem. Biophys. Res. Commun. 34:555–559.
Bartels, P. D., and Sestoft, L., 1980, Thyroid hormone induced changes in gluconeogenesis and ketogenesis in perfused rat liver, Biochim. Biophys. Acta 633:56–57.
Bartlett, K., Bone, A. J., Koundajian, P., Meredith, P., Turnbull, D. M., and Sherratt, H. S. A., 1981, Inhibition of mitochondrial β-oxidation at the stage of carnitine palmitoyltransferase I by the coenzyme A esters of some substituted hypoglycaemic oxiran-2-carboxylic acids, Biochem. Soc. Trans. 9:574–575.
Benito, M., and Williamson, D. H., 1978, Evidence for a reciprocal relationship between lipogenesis and ketogenesis in hepatocytes from fed virgin and lactating rats, Biochem. J. 176:331–334.
Benito, M., Whitelaw, E., and Williamson, D. H., 1979, Regulation of ketogenesis during the suckling- weanling transition in the rat, Biochem. J. 180:137–144.
Berge, R. K., and Broch, O. J., 1981, Regional and subcellular distribution of acyl-CoA hydrolase and acyl-1-carnitine hydrolase in young and adult rat brain, Int. J. Biochem. 13:1157–1162.
Berge, R. K., Slinde, E., and Farstad, M., 1979, Intracellular localization of long-chain acyl-coenzyme A hydrolase and acyl-1-carnitine hydrolase in brown adipose tissue from guinea pigs, Biochim. J. 182:347–351.
Bergstrom, J. D., and Reitz, R. C., 1980, Studies on carnitine palmitoyltransferase: The similar nature of CPTi (inner form) and CPT0 (outer form), Arch. Biochem. Biophys. 204:71–79.
Bieber, L. L., Abraham, T., and Helmrath, T., 1972, A rapid spectrophotometric assay for carnitine palmitoyltransferase, Anal. Biochem. 50:509–518.
Bieber, L. L., Markwell, M. A. K., Blair, M., and Helmrath, T. A., 1973, Studies on the development of carnitine palmitoyltransferase and fatty acid oxidation in liver mitochondria of neonatal pigs, Biochem. Biophys. Acta 326:145–154.
Borrebaek, B., Osmundsen, H., and Bremer, J., 1980, In vivo induction of 4-enoyl-CoA reductase by clofibrate in liver mitochondria and its effect on pent-4-enoate metabolism, Biochem. Biophys. Res. Commun. 93:1173–1180.
Brady, L. J., and Hoppel, C. L., 1983a, Hepatic mitochondrial function in lean and obese Zucker rats, Am. J. Physiol., 245:E239–E245.
Brady, L. J., and Hoppel, C. L., 1983b, Effect of diet and starvation on hepatic mitochondrial function in the rat, J. Nutr., 113:2129–2137.
Brass, E. P., and Hoppel, C. L., 1978a, Carnitine metabolism in the fasting rat, J. Biol. Chem. 253:2688–2693.
Brass, E. P., and Hoppel, C. L., 1978b, Disassociation between acid insoluble acylcarnitines and ketogenesis following carnitine administration in vivo, J. Biol. Chem. 253:5274–5276.
Brass, E. P., and Hoppel, C. L., 1980, Effect of carnitine on mitochondrial oxidation of palmitoylcarnitine, Biochem. J. 188:451–458.
Bremer, J., 1962, Carnitine in intermediary metabolism. The metabolism of fatty acid esters of carnitine by mitochondria, J. Biol. Chem. 237:3628–3632.
Bremer, J., 1963, Carnitine in intermediary metabolism. The biosynthesis of palmityl-carnitine by cell subfractions, J. Biol. Chem. 238:2774–2779.
Bremer, J., 1967, The function of palmityl-CoA:carnitine palmityltransferase in the oxidation of fatty acids, in:Protides of the Biological Fluids, Vol. 15 ( H. Peeters, ed.), Elsevier, Amsterdam, pp. 185–189.
Bremer, J., 1981, The effect of fasting on the activity of liver carnitine palmitoyltransferase and its inhibition by malonyl-CoA, Biochim. Biophys. Acta 665:628–631.
Bremer, J., and Norum, K. R., 1967a, The mechanism of substrate inhibition of palmityl coenzyme A:carnitine palmityltransferase by palmityl-coenzyme A, J. Biol. Chem. 242:1744–1748.
Bremer, J., and Norum, K. R., 1967b, The effects of detergents on palmityl coenzyme A:carnitine palmityltransferase, J. Biol. Chem. 242:1749–1755.
Bremer, J., and Norum, K. R., 1967c, Palmityl-CoA:carnitine-o-palmityltransferase in the mitochondrial oxidation of palmityl-CoA, Eur. J. Biochem. 1:427–433.
Bremer, J., and Wojtczak, A. B., 1972, Factors controlling the rate of fatty acid beta-oxidation in rat liver mitochondria, Biochim. Biophys. Acta 280:515–530.
Brosnan, J. T., and Fritz, I. B., 1971a, The permeability of mitochondria to carnitine and acetylcarnitine, Biochem. J. 125:94 P.
Brosnan, J. T., and Fritz, I. B., 1971b, The oxidation of fatty-acyl derivatives by mitochondria from bovine fetal and calf hearts, Can. J. Biochem. 49:1296–1300.
Brosnan, J. T., Kopec, B., and Fritz, I. B., 1973, The localization of carnitine palmitoyltransferase on the inner membrane of bovine liver mitochondria, J. Biol. Chem. 248:4075–4082.
Cederbaum, A. I., Lieber, C. S., Beattie, D. S., and Rubin, E., 1975, Effect of chronic ethanol ingestion on fatty acid oxidation by hepatic mitochondria, J. Biol. Chem. 250:5122–5129.
Chase, J. F. A., and Tubbs, P. K., 1972, Specific inhibition of mitochondrial fatty acid oxidation by 2- bromopalmitate and its coenzyme A and carnitine esters, Biochem. J. 129:55–65.
Christiansen, R. Z., 1977, Regulation of palmitate metabolism by carnitine and glucagon in hepatocytes isolated from fasted and carbohydrate refed rats, Biochim. Biophys. Acta 488:249–262.
Christiansen, R. Z., 1978, The effect of clofibrate-feeding on hepatic fatty acid metabolism, Biochim. Biophys. Acta 530:314–324.
Christiansen, R. Z., Osmundsen, H., Borrebaeck, B., and Bremer, J., 1978, The effects of clofibrate feeding on the metabolism of palmitate and erucate in isolated hepatocytes, Lipids 13:487–491.
Ciardelli, T., Stewart, C. J., Seeliger, A., and Wieland, T., 1981, Synthesis of a carba-analog of S-palmitoyl-coenzyme-A, heptadecan-2-onyldethio-CoA, and of 5-heptadecyl-CoA; effective inhibitors of citrate synthase and carnitine palmitoyltransferase, Liebigs Ann. Chem. 1981:828–841.
Clarke, P. R. H., and Bieber, L. L., 1981a, Isolation and purification of mitochondrial carnitine octanoyltransferase activities from beef heart, J. Biol. Chem. 256:9861–9868.
Clarke, P. R. H., and Bieber, L. L., 1981b, Effect of micelles on the kinetics of purified beef heart mitochondrial carnitine palmitoyltransferase, J. Biol. Chem. 256:9869–9873.
Cook, G. A., Otto, D. A., and Cornell, N. W., 1980, Differential inhibition of ketogenesis by malonyl-CoA in mitochondria from fed and starved rats, Biochem. J. 192:955–958.
Daae, L. N. W., and Aas, M., 1973, Fatty acid activation and acyl transfer in rat liver during clofibrate feeding, Atherosclerosis 17:389–400.
Delisle, G., and Fritz, I. B., 1967, Interrelations between hepatic fatty acid oxidation and gluconeogenesis:A possible regulatory role of carnitine palmityltransferase, Proc. Natl. Acad. Sci. USA 58:790–797.
Demaugre, F., Buc, H., Girard, J., and Leroux, J. P., 1983, Role of the mitochondrial metabolism of pyruvate on the regulation of ketogenesis in rat hepatocytes, Metabolism 32:40–48.
DiMarco, J. P., and Hoppel, C., 1975, Hepatic mitochondrial function in ketogenic states. Diabetes, starvation, and after growth hormone administration, J. Clin. Invest. 55:1237–1244.
DiMauro, S., and Trevisan, C., 1982, Carnitine palmitoyltransferase (CPT) deficiency:A review, in:Disorders of the Motor Unit ( D. L. Schotland, ed.), John Wiley and Sons, New York, pp. 657–666.
Farrell, S. O., and Bieber, L. L., 1983, Carnitine octanoyltransferase of mouse liver peroxisomes:Properties and effect of hypolipidemic drugs, Arch. Biochem. Biophys. 222:123–132.
Ferre, P., Pegorier, J. P., Williamson, D. H., and Girard, J. R., 1978, The development of ketogenesis at birth in the rat, Biochem. J. 176:759–765.
Ferri, L., Jocelyn, P. C., and Siliprandi, N., 1980, The mitochondrial handling of D,L-thiocarnitine and its s-acetyl derivative, FEBS Lett. 121:19–22.
Foster, P. C., and Bailey, E., 1976a, Changes in hepatic fatty acid degradation and blood lipid and ketone body content during development of the rat, Enzyme 21:397–407.
Foster, P. C., and Bailey, E., 1976b, Changes in the activities of the enzymes of hepatic fatty acid oxidation during development of the rat, Biochem. J. 154:49–56.
Fritz, I. B., 1955, The effects of muscle extracts on the oxidation of palmitic acid by liver slices and homogenates, Acta Physiol. Scand. 34:367–385.
Fritz, I. B., 1957, Effects of choline deficiency and carnitine on palmitic acid oxidation by rat liver homogenates, Am. J. Physiol. 190:449–452.
Fritz, I. B., 1959, Action of carnitine on long chain fatty acid oxidation by liver, Am. J. Physiol. 197:297–304.
Fritz, I. B., 1961, Factors influencing the rates of long-chain fatty acid oxidation and synthesis in mammalian systems, Physiol. Rev. 41:52–129.
Fritz, I. B., 1967, An hypothesis concerning the role of carnitine in the control of intermediates between fatty acid and carbohydrate metabolism, Perspect. Biol. Med. 10:643–677.
Fritz, I. B., and Marquis, N. R., 1965, The role of acylcarnitine esters and carnitine palmityltransferase in the transport of fatty acyl groups across mitochondrial membranes, Proc. Natl. Acad. Sci. USA 54:1226–1233.
Fritz, I. B., and Yue, K. T. N., 1963, Long-chain carnitine acyltransferase and the role of acylcarnitine derivatives in the catalytic increase of fatty acid oxidation induced by carnitine, J. Lipid Res. 4:279–288.
Fritz, I. B., Kaplan, E., and Yue, K. T. N., 1962, Specificity of carnitine action on fatty acid oxidation by heart muscle, Am. J. Physiol. 202:117–121.
Garland, P. B., Haddock, B. A., and Yates, D. W., 1969, Components and compartments of mitochondrial fatty acid oxidation, in:FEBS Symp. 17 ( L. Ernster and Z. Drahota, eds.), Academic Press, London, pp. 111–126.
Haddock, B. A., Yates, D. W., and Garland, P. D., 1970, The localization of some coenzyme A-dependent enzymes in rat liver mitochondria, Biochem. J. 119:565–573.
Hagen, L. E., Berge, R. K., and Farstad, M., 1979, Different subcellular localization of palmitoyl-1-carnitine hydrolysis in human and rat liver, FEBS Lett. 104:297–299.
Hahn, P., 1981, Serum carnitine levels and hepatic and adipose tissue carnitine transferases in obese mice, Nutr. Res. 1:93–99.
Halperin, M. L., and Pande, S. V., 1979, Fatty acyl group transport into mitochondria:Carnitine palmitoyltransferase EC 2.3.1.23 and the carnitine-acylcarnitine translocase, Meth. Enzymol. 56:368–378.
Harano, Y., Kowal, J., Yamazaki, R., Lavine, L., and Miller, M., 1972, Carnitine palmitoyltransferase activities (1 and 2) and the rate of palmitate oxidation in liver mitochondria from diabetic rats, Arch. Biochim. Biophys. 153:426–437.
Harano, Y., Kosugi, K., Kashiwagi, A., Nakano, T., Hidaka, H., and Shigeta, Y., 1982, Regulatory mechanism of ketogenesis by glucagon and insulin in isolated and cultured hepatocytes, J. Biochem. 91:1739–1748.
Hoppel, C. L., 1976, Carnitine palmitoyltransferase and transport of fatty acids, in:The Enzymes of Biological Membranes, Vol. 2 ( A. Martonosi, ed.), Plenum Press, New York, pp. 119–143.
Hoppel, C. L., and Tomec, R. J., 1972, Carnitine palmityltransferase. Location of two enzymatic activities in rat liver mitochondria, J. Biol. Chem. 247:832–844.
Hoppel, C. L., Genuth, S., Brass, E., Fuller, R., and Hostetler, K., 1980, Carnitine and carnitine palmitoyltransferase in metabolic studies, in:Carnitine Biosynthesis, Metabolism, and Functions ( R. A. Frenkel and J. D. McGarry, eds.), Academic Press, New York, pp. 287–305.
Hostetler, K. Y., Hoppel, C. L., Romine, J. S., Sipe, J. C., Gross, S. R., and Higginbottom, P. A., 1978, Partial deficiency of muscle carnitine palmitoyltransferase with normal ketone production, New Engl. J. Med. 298:553–557.
Idell-Wenger, J. A., 1981, Carnitine:acetylcarnitine translocase of rat heart mitochondria. Competition for carnitine uptake by carnitine esters, J. Biol. Chem. 256:5597–5603.
Kahonen, M. T., 1976, Effect of clofibrate treatment on carnitine acyltransferases in different subcellular fractions of rat liver, Biochim. Biophys. Acta 428:690–701.
Kahonen, M. T., 1979, Effect of clofibrate treatment on acylcarnitine oxidation in isolated rat liver mitochondria, Med. Biol. 67:58–65.
Keyes, W. G., and Heimberg, M., 1979, Influence of thyroid status on lipid metabolism in the perfused rat liver, J. Clin. Invest. 64:182–190.
Keyes, W. G., Wilcox, H. G., and Heimberg, M., 1981, Formation of the very low density lipoproteins and metabolism of [l-14C]oleate by perfused livers from rats treated with triiodothyronine or propylthiouracil, Metabolism 30:135–146.
Kopec, B., and Fritz, I. B., 1971, Properties of a purified carnitine palmitoyltransferase, and evidence for the existence of other carnitine acyltransferases, Can. J. Biochem. 49:941–948.
Kopec, B., and Fritz, I. B., 1973, Comparison of properties of carnitine palmitoyltransferase I with those of carnitine palmitoyltransferase II, and preparation of antibodies to carnitine palmitoyltransferases, J. Biol. Chem. 248:4069–4074.
Kurup, C. K. R., Aithal, H. N., and Ramasarma, T., 1970, Increase in hepatic mitochondria on administration of ethyl-α-p-chlorophenoxy isobutyrate to the rat, Biochem. J. 116:773–779.
Laker, M. E., and Mayes, P. A., 1981, Effect of hyperthroidism and hypothyroidism on lipid and carbohydrate metabolism of the perfused rat liver, Biochem. J. 196:247–255.
Levitsky, D. O., and Skulachev, V. P., 1972, Carnitine:The carrier transporting fatty acids into mitochondria by means of an electrochemical gradient of H+, Biochim. Biophys. Acta 275:33–50.
Lockwood, E. A., and Bailey, E., 1970, Fatty acid utilization during development of the rat, Biochem. J. 120:49–54.
Mahadevan, S., and Sauer, F., 1969, Carnitine ester hydrolase of rat liver, J. Biol. Chem. 244:4448–4453.
Mannaerts, G. P., Thomas, J., Debeer, L. J., McGarry, J. D., and Foster, D. W., 1978, Hepatic fatty acid oxidation and ketogenesis after clofibrate treatment, Biochim. Biophys. Acta 529:201–211.
Markwell, M. A. K., McGroarty, E. J., Bieber, L. L., andTolbert, N. E., 1973, The subcellular distribution of carnitine acyltransferases in mammalian liver and kidney. A new peroxisomal enzyme, J. Biol. Chem. 248:3426–3432.
Markwell, M. A. K., Tolbert, N. E., and Bieber, L. L., 1976, Comparison of the carnitine acyltransferase activities from rat liver peroxisomes and microsomes, Arch. Biochem. Biophys. 176:479–488.
McCune, S. A., Durant, P. J., Jenkins, P. A., and Harris, R. A., 1981, Comparative studies on fatty acid synthesis, glycogen metabolism, and gluconeogenesis by hepatocytes isolated from lean and obese Zucker rats, Metabolism 30:1170–1178.
McGarry, J. D., and Foster, D. W., 1973, Acute reversal of experimental diabetic ketoacidosis in the rat with (+)-decanoylcarnitine, J. Clin. Invest. 52:877–884.
McGarry, J. D., and Foster, D. W., 1979, In support of the roles of malonyl-CoA and carnitine acyltransferase I in the regulation of hepatic fatty acid oxidation and ketogenesis, J. Biol. Chem. 254:8163–8168.
McGarry, J. D., and Foster, D. W., 1980, Regulation of hepatic fatty acid oxidation and ketone production, Annu. Rev. Biochem. 49:395–420.
McGarry, J. D., and Foster, D. W., 1981, Importance of experimental conditions in evaluating the malonyl-CoA sensitivity of liver carnitine acyltransferase, Biochem. J. 200:217–223.
McGarry, J. D., and Foster, D. W., 1982, Malonyl-CoA inhibition of carnitine palmitoyltransferase I:Response to Dr. Saggerson’s letter, Biochem. J. 208:527–528.
McGarry, J. D., Meier, J. M., and Foster, D. W., 1973, The effects of starvation and refeeding on carbohydrate and lipid metabolism in vivo and in the perfused rat liver. The relationship between fatty acid oxidation and esterification in the regulation of ketogenesis, J. Biol. Chem. 248:270–278.
McGarry, J. D., Robles-Valdes,C., and Foster, D. W., 1975, Role of carnitine in hepatic ketogenesis, Proc. Natl. Acad. Sci. USA 72:4385–4388.
McGarry, J. D., Mannaerts, G. P., and Foster, D. W., 1977, A possible role for malonyl-CoA in the regulation of hepatic fatty acid oxidation and ketogenesis, J. Clin. Invest. 60:265–270.
McGarry, J. D., Leatherman, G. F., and Foster, D. W., 1978a, Carnitine palmitoyltransferase I. The site of inhibition of hepatic fatty acid oxidation by malonyl-CoA, J. Biol. Chem. 253:4128–4136.
McGarry, J. D., Takabayashi, Y., and Foster, D. W., 1978b, The role of malonyl-CoA in the coordination of fatty acid synthesis and oxidation in isolated rat hepatocytes, J. Biol. Chem. 253:8294–8300.
Murray, W. J., Reed, K. W., and Roche, E. B., 1980, Conformations of carnitine and acetylcarnitine and the relationship of mitochondrial transport of fatty acids, J. Theoret. Biol. 82:559–572.
Normann, P. T., and Flatmark, T., 1978, Long-chain acyl-CoA synthetase and “outer” carnitine long-chain acyltransferase activities of intact brown adipose tissue mitochondria, Biochim. Biophys. Acta 530:461–473.
Normann, P. T., Ingebretsen, O. C., and Flatmark, T., 1978, On the rate-limiting step in the transfer of long-chain acyl groups across the inner membrane of brown adipose tissue mitochondria, Biochim. Biophys. Acta 501:286–295.
Norum, K. R., 1964, Palmityl-CoA:carnitine palmityltransferase purification from calf-liver mitochondria and some properties of the enzyme, Biochim. Biophys. Acta 89:95–108.
Norum, K. R., 1965a, Activation of palmityl-CoA:carnitine palmityltransferase in livers from fasted, fat-fed, or diabetic rats, Biochim. Biophys. Acta 98:652–654.
Norum, K. R., 1965b, Palmityl-CoA:carnitine palmityltransferase. Studies on the substrate specificity of the enzyme, Biochim. Biophys. Acta 99:511–522.
Norum, K. R., and Bremer, J., 1967, The localization of acyl coenzyme A-carnitine acyltransferases in rat liver cells, J. Biol. Chem. 242:407–411.
Norum, K. R., Farstad, M., and Bremer, J., 1966, The submitochondrial distribution of acid:CoA ligase (AMP) and palmityl-CoA:carnitine palmityltransferase in rat liver mitochondria, Biochem. Biophys. Res. Commun. 24:797–804.
Nosadini, R., Ursini, F., Tessari, P., Giengo, A., and Gregolin, C., 1979, Perfused liver carnitine palmitoyltransferase activity and ketogenesis in streptozotocin treated and genetic hyperinsulinemic rats. Effect of glucagon, Horm. Metabol. Res. 11:661–664.
Ontko, J. A., and Johns, M. L., 1980, Evaluation of malonyl-CoA in the regulation of long-chain fatty acid oxidation in the liver. Evidence for an unidentified regulatory component of the system, Biochem. J. 192:959–962.
Osmundsen, H., Cervenka, J., and Bremer, J., 1982, A role for 2,4-enoyl-CoA reductase in mitochondrial β-oxidation of polyunsaturated fatty acids. Effects of treatment with clofibrate on oxidation of polyunsaturated acylcarnitines by isolated rat liver mitochondria, Biochem. J. 208:749–757.
Pande, S. V., 1971, On rate controlling factors of long chain fatty acid oxidation, J. Biol. Chem. 246:5384–5390.
Pande, S. V., 1975, A mitochondrial carnitine acylcarnitine translocase system, Proc. Natl. Acad. Sci. USA 72:883–887.
Pande, S. V., and Parvin, R., 1976, Characterization of carnitine acylcarnitine translocase system of heart mitochondria, J. Biol. Chem. 251:6683–6691.
Pande, S. V., and Parvin, R., 1980a, Carnitine-acylcarnitine translocase catalyzes an equilibrating unidirectional transport as well, J. Biol. Chem. 255:2994–3001.
Pande, S. V., and Parvin, R., 1980b, Clofibrate enhancement of mitochondrial carnitine transport system of rat liver and augmentation of liver carnitine and γ-butyrobetaine hydroxylase activity by thyroxine, Biochim. Biophys. Acta 617:363–370.
Parker, S. L., Thompson, J. A., and Reitz, R. C., 1974, Effects of chronic ethanol ingestion upon acyl-CoA:carnitine acyltransferase in liver and heart, Lipids 9:520–525.
Parvin, R., and Pande, S. V., 1979, Enhancement of mitochondrial carnitine and carnitine acylcarnitine translocase-mediated transport of fatty acids into liver mitochondria under ketogenic conditions, J. Biol. Chem. 254:5423–5429.
Parvin, R., Goswami, T., and Pande, S. V., 1980, Inhibition of mitochondrial carnitine-acylcarnitine translocase by sulfobetaines, Can. J. Biochem. 58:822–830.
Patten, B. M., Wood, J. M., Harati, Y., Hefferan, P., and Howell, R. R., 1979, Familial recurrent rhabdomyolysis due to carnitine palmityltransferase deficiency, Am. J. Med. 67:167–171.
Powell, G. L., Grothusen, J. R., Zimmerman, J. K., Evans, C. A., and Fish, W. W., 1981, A reexamination of some properties of fatty acyl-CoA micelles, J. Biol. Chem. 256:12740–12747.
Ramsay, R. R., and Tubbs, P. K., 1974, Exchange of the endogenous carnitine of ox heart mitochondria with external carnitine and its possible relevance to the mechanism of fatty-acyl transport into mitochondria, Biochem. Soc. Trans. 2:1285–1286.
Ramsay, R. R., and Tubbs, P. K., 1975, The mechanism of fatty acid uptake by heart mitochondria:An acylcarnitine-carnitine exchange, FEBS Lett. 54:21–25.
Ramsay, R. R., and Tubbs, P. K., 1976, The effects of temperature and some inhibitors on the carnitine exchange system of heart mitochondria, Eur. J. Biochem. 69:299–303.
Robinson, L. N., and Zammit, V. A., 1982, Sensitivity of carnitine acyltransferase I to malonyl-CoA inhibition in isolated rat liver mitochondria is quantitatively related to hepatic malonyl-CoA concentration in vivo, Biochem. J. 206:177–179.
Robles-Valdes, C., McGarry, J. D., and Foster, D. W., 1976, Maternal-fetal carnitine relationships and neonatal ketosis in the rat, J. Biol. Chem. 251:6007–6012.
Saggerson, E. D., 1982, Does fasting decrease the inhibitory effect of malonyl-CoA on hepatic β-oxidation? Biochem. J. 208:525–526.
Saggerson, E. D., and Carpenter, C. A., 1981a, Effects of fasting, adrenalectomy and streptozotocin-diabetes on sensitivity of hepatic carnitine acyltransferase to malonyl-CoA, FEBS Lett. 129:225–228.
Saggerson, E. D., and Carpenter, C. A., 1981b, Carnitine palmitoyltransferase and carnitine octanoyltransferase activities in liver, kidney cortex, adipocyte, lactating mammary gland, skeletal muscle and heart; relative activities, latency, and effect of malonyl-CoA, FEBS Lett. 129:229–232.
Saggerson, E. D., and Carpenter, C. A., 1981c, Effects of fasting and malonyl-CoA on the kinetics of carnitine palmitoyltransferase and carnitine octanoyltransferase in intact rat liver mitochondria, FEBS Lett. 132:166–168.
Saggerson, E. D., and Carpenter, C. A., 1982, Regulation of hepatic carnitine palmitoyltransferase activity during the foetal-neonatal transition, FEBS Lett. 150:177–180.
Saggerson, E. D., and Carpenter, C. A., 1983, The effect of malonyl-CoA on overt and latent carnitine acyltransferase activities in rat liver and adipocyte mitochondria, Biochem. J. 210:591–597.
Saggerson, E. D., Carpenter, C. A., and Tselentis, B. S., 1982, Effects of thyroidectomy and starvation on the activity and properties of hepatic carnitine palmitoyltransferase, Biochem. J. 208:667–672.
Scholte, H. R., Jennekens, F. G. I., and Bouvy, J. J. B. J., 1979, Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leukocytes, J. Neurol. Sci. 40:39–51.
Schulz, H., and Racker, E., 1979, Carnitine transport in submitochondrial particles and reconstituted proteoliposomes, Biochem. Biophys. Res. Commun. 89:134–140.
Solberg, H. E., 1972, Different carnitine acyltransferases in calf liver, Biochim. Biophys. Acta 280:422–433.
Solberg, H. E., 1974, Acyl group specificity of mitochondrial pool of carnitine acyltransferases, Biochim. Biophys. Acta 360:101–112.
Solberg, H. E., Aas, M., and Daae, L. N. W., 1972, The activity of the different carnitine acyltransferases in the liver of clofibrate-fed rats, Biochim. Biophys. Acta 280:434–439.
Stakkestad, J. A., and Bremer, J., 1982, The metabolism of fatty acids in hepatocytes isolated from triiodothyronine-treated rats, Biochim. Biophys. Acta 711:90–100.
Stakkestad, J. A., and Bremer, J., 1983, The outer carnitine palmitoyltransferase and regulation of fatty acid metabolism in rat liver in different thyroid states, Biochem. Biophys. Acta, 750:244–252.
Stanley, C. A., Gonzales, E., and Baker, L., 1983, Development of hepatic fatty acid oxidation and ketogenesis in the newborn guinea pig, Pediat. Res. 17:224–229.
Tomec, R. J., and Hoppel, C. L., 1975, Carnitine palmitoyltransferase in bovine fetal heart mitochondria, Arch. Biochem. Biophys. 170:716–723.
Triscari, J., Greenwood, M. R. C., and Sullivan, A. C., 1982, Oxidation and ketogenesis in hepatocytes of lean and obese Zucker rats, Metabolism 31:223–228.
Tubbs, P. K., and Chase, J. F. A., 1970, Effects of an acylcarnitine analogue, 2-bromomyristoyl-thiocarnitine, on mitochondrial respiration, Biochem. J. 116:34 p.
Tubbs, P. K., Ramsay, R. R., and Edwards, M. R., 1980, Inhibitors of carnitine transport and metabolism, in:Carnitine Biosynthesis, Metabolism, and Functions ( R. A. Frenkel and J. D. McGarry, eds.), Academic Press, New York, pp. 207–217.
Tutwiler, G. F., and Dellevigne, P., 1979, Action of the oral hypoglycemic agent 2-tetradecylglycidic acid on hepatic fatty acid oxidation and gluconeogenesis, J. Biol. Chem. 254:2935–2941.
Tutwiler, G. F., and Ryzlak, M. T., 1980, Inhibition of mitochondrial carnitine palmitoyltransferase by 2-tetradecylglycidic acid (McN-3802), Life Sci. 26:393–397.
Tutwiler, G. F., Ho, W., and Mahrbrachr, R. J., 1981, Tetradecylglycidic acid, Meth. Enzymol. 72:533–551.
VanTol, A., 1974, The effect of fasting on the acylation of carnitine and glycerophosphate in rat liver subcellular fractions, Biochim. Biophys. Acta 357:14–23.
VanTol, A., and Hulsmann, W. C., 1969, The localization of palmitoyl –CoA:carnitine palmitoyltransferase in rat liver, Biochim. Biophys. Acta 189:342–353.
Warshaw, J. B., 1972, Cellular energy metabolism during fetal development. IV. Fatty acid activation, acyl transfer and fatty acid oxidation during development of the chick and rat, Dev. Biol. 28:537–544.
Warshaw, J. B., and Terry, M. L., 1970, Cellular energy metabolism during fetal development. II. Fatty acid oxidation by the developing heart. J. Cell. Biol. 44:354–360.
West, D. W., Chase, J. F. A., and Tubbs, P. K., 1971, The separation and properties of two forms of carnitine palmitoyltransferase from ox liver mitochondria, Biochem. Biophys. Res. Commun. 42:912–918.
Williams, E. S., and Li, T.-K., 1977, The effect of chronic alcohol administration on fatty acid metabolism and pyruvate oxidation of heart mitochondria, J. Mol. Cell. Cardiol. 9:1003–1011.
Williamson, J. R., Browning, E. T., Scholz, R., Kreisberg, R. A., and Fritz, I. B., 1968, Inhibition of fatty acid stimulation of gluconeogenesis by (+)-decanoylcarnitine in perfused rat liver, Diabetes 17:194–208.
Wittels, B., and Bressler, R., 1965, Lipid metabolism in the newborn rat, J. Clin. Invest. 44:1639–1646.
Wolkowicz, P. E., Pownall, H. J., and McMillin-Wood, J. B., 1982, (1-Pyrenebutyryl)carnitine and 1-pyrenebutyryl coenzyme A:Fluorescent probes for lipid metabolite studies in artificial and natural membranes, Biochemistry 21:2990–2996.
Wood, J. McM., 1973, Effect of ionic strength on the activity of carnitine palmityltransferase I, Biochemistry 12:5268–5273.
Wood, J. McM., Sordahl, L. A., Lewis, R. M., and Schwartz, A., 1973, Effect of chronic myocardial ischemia on the activity of carnitine palmitylcoenzyme A transferase of isolated canine heart mitochondria, Circ. Res. 32:340–347.
Yalkowsky, S. H., and Zografi, G., 1970, Potentiometric titration of monomeric and micellar acylcarnitines, J. Pharm. Sci. 59:798–802.
Yates, D. W., and Garland, P. B., 1966, The partial latency and intramitochondrial distribution of carnitine palmitoyltransferase (EC 2.3.1.-), and the CoASH and carnitine permeable space of rat liver mitochondria, Biochem. Biophys. Res. Commun. 23:460–465.
Yates, D. W., and Garland, P. B., 1970, Carnitine palmitoyltransferase activities (EC 2.3.1.-) of rat liver mitochondria, Biochem. J. 119:547–552.
Yeh, Y. Y., and Yee, P., 1976, Insulin, a possible regulator of ketosis in newborn and suckling rats, Pediat. Res. 10:192–197.
Yeh, Y.-Y., and Yee, P., 1979, Fatty acid oxidation in isolated rat liver mitochondria. Developmental changes and their relation to hepatic levels of carnitine and glycogen and to carnitine acyltransferase activity, Arch. Biochem. Biophys. 199:560–569.
Zahler, W. L., Barden, R. E., and Cleland, W. W., 1968, Some physical properties of palmitoyl-coenzyme A micelles, Biochim. Biophys. Acta 164:1–11.
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© 1985 Plenum Press, New York
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Hoppel, C.L., Brady, L. (1985). Carnitine Palmitoyltransferase and Transport of Fatty Acids. In: Martonosi, A.N. (eds) The Enzymes of Biological Membranes. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-2355-6_4
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