Serum carnitine concentrations in patients with idiopathic hypertrophic cardiomyopathy: Relationship with impaired myocardial fatty acid metabolism

Clinical Science

Volumn 97, Issue 4, 1999, Pages 493-501

  Nakamura, Tomoki ^a   Sugihara, Hiroki ^a   Kinoshita, Noriyuki ^a   Ito, Kazuki ^a   Adachi, Yoshihiko ^a   Hirasaki, Satoshi ^a   Matsuo, Akiko ^a   Azuma, Akihiro ^a   Kodo, Naoki ^a   Nakagawa, Masao ^a  

^a KYOTO PREFECTURAL UNIVERSITY OF MEDICINE   (Japan)

Author keywords

123I 15 (p iodophenyl) 3 R,S methylpentadecanoic acid (BMIPP); Carnitine; Fatty acid metabolism; Hypertrophic cardiomyopathy

Indexed keywords

ACYLCARNITINE; CARNITINE;

ADOLESCENT; ADULT; AGED; ARTICLE; BLOOD LEVEL; CARNITINE DEFICIENCY; CONTROLLED STUDY; FATTY ACID METABOLISM; FEMALE; HEART CATHETERIZATION; HEART EJECTION FRACTION; HUMAN; HYPERTROPHIC CARDIOMYOPATHY; ISOTOPE LABELING; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; SCINTIGRAPHY;

ADOLESCENT; ADULT; AGED; BIOLOGICAL MARKERS; CARDIOMYOPATHY, HYPERTROPHIC; CARNITINE; FATTY ACIDS; FATTY ACIDS, NONESTERIFIED; FEMALE; HUMANS; IODOBENZENES; MALE; MIDDLE AGED; MYOCARDIUM;

EID: 0032828536     PISSN: 01435221     EISSN: None     Source Type: Journal    
DOI: 10.1042/CS19990066     Document Type: Article

References (43)

1. 1 Bing, R. J. (1965) Cardiac metabolism. Physiol Rev. 45, 171-213
2. 2 Neeley, J. R. and Morgan, H. E. (1974) Relationship between carbohydrate and lipid metabolism and the energy balance of heart muscle. Annu. Rev. Physiol. 36, 413-459
3. 3 Lopaschuk, G. D., Belke, D. D., Gamble, J., Itoi, T. and Schonekess, B. O. (1994) Regulation of fatty acid oxidation in the mammalian heart in health and disease. Biochem. Biophys. Acta 1213, 263-276
4. 4 Engel, A. G. (1980) Possible causes and effects of carnitine deficiency in man. In Carnitine Biosynthesis, Metabolism and Function (Frenkel, R. A. and McGarry, J. D., eds.), pp. 271-285, Academic Press, New York
5. 5 Bremer, J. (1983) Carnitine - metabolism and functions. Physiol. Rev. 63, 1420-1442
6. 6 Vary, T. C. and Neely, J. R. (1982) Characterization of carnitine transport in isolated perfused adult rat hearts. Am. J. Physiol. 242, H585-H592
7. 7 Kurata, C., Tawarahara, K., Taguchi, T. et al. (1992) Myocardial emission computed tomography with iodine-123-labelled beta-methyl-branched fatty acid in patients with hypertrophic cardiomyopathy. J. Nucl. Med. 33, 6-13
8. 8 Tadamura, E., Kudoh, T., Hattori, N. et al. (1998) Impairment of BMIPP uptake precedes abnormalities in oxygen and glucose metabolism in hypertrophic cardiomyopathy. J. Nucl. Med. 39, 390-396
9. 123I-BMIPP myocardial single photon emission computed tomography. Ann. Nucl. Med. 10, 71-78
10. 10 Regitz, V. R., Shug, A. L. and Fleck, E. (1990) Defective myocardial carnitine metabolism in congestive heart failure secondary to dilated cardiomyopathy and coronary, hypertensive, and valvular diseases. Am. J. Cardiol. 65, 755-760
11. 11 Tripp, M. E. and Shug, A. L. (1984) Plasma carnitine concentrations in cardiomyopathy patients. Biochem. Med. 32, 199-206
12. 12 Maron, B. J. and Epstein, S. E. (1979) Hypertrophic cardiomyopathy. A discussion of nomenclature. Am. J. Cardiol. 43, 1242-1244
13. 13 WHO/ISFC Task Force (1980) Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies. Br. Heart J. 44, 672-673
14. 14 Tateno, M., Tamaki, N., Yukihiro, M. et al. (1996) Assessment of fatty acid uptake in ischemic heart disease without myocardial infarction. J. Nucl. Med. 37, 1981-1985
15. 15 Bohmer, T. and Molstad, P. (1980) Carnitine transport across the plasma membrane. In Carnitine Biosynthesis, Metabolism, and Functions (Frenkel, R. A. and McGarry, J. D., eds.), pp. 73-89, Academic Press, New York
16. 16 Chen, S. and Lincoln, S.D. (1977) Increased serum carnitine concentration in renal insufficiency. Clin. Chem. 23, 278-280
17. 17 Kanda, T., Suzuki, Y., Toyama, T. et al. (1995) Myocardial uptake of an iodinated branched fatty acid analog, assessed by SPECT, may detect metabolic derangement of the myocardium in diabetic patients with coronary heart disease. Cardiology 86, 238-242
18. 18 Takahashi, M., Ueda, S. and Misaki, H. (1992) A highly sensitive method for determination of serum L-carnitine and acylcarnitine using an enzymatic cycling reaction. Clin. Chem. 38, 958-959
19. 19 Takahashi, M., Ueda, S., Misaki, H. et al. (1994) Carnitine determination by an enzymatic cycling method with carnitine dehydrogenase. Clin. Chem. 40, 817-821
20. 20 Uji, Y., Okabe, H., Sakai, Y., Noma, A., Maeda, M. and Tsuji, A. (1989) Chemiluminescent determination of nonesterified fatty acids in serum. Clin. Chem. 35, 325-326
21. 21 Nakamura, T., Sugihara, H., Inaba, T. et al. (1999) CD36 deficiency has little influence on the pathophysiology of hypertrophic cardiomyopathy. J. Mol. Cell. Cardiol. 31, 1253-1259
22. 22 Sahn, D. J., DeMaria, A., Kisslo, J. and Weyman, A. (1978) Recommendations regarding quantitation in M-mode echocardiography : results of a survey of echocardiographic measurements. Circulation 58, 1072-1083
23. 23 Cantrell, C. R. and Borum, P. R. (1982) Identification of a cardiac carnitine binding protein. J. Biol. Chem. 257, 10599-10604
24. 24 Bohmer, T., Eiklid, K. and Jonsen, J. (1977) Carnitine uptake into human heart cells in culture. Biochim. Biophys. Acta 465, 627-633
25. 25 Siliprandi, N., Ciman, M. and Sartorelli, L. (1987) Myocardial carnitine transport. Basic Res. Cardiol. 82, 53-62
26. 26 Idell-Wenger, J. A., Grotyohann, L. W. and Neely, J. R. (1977) Coenzyme A and carnitine distribution in normal and ischemic hearts. J. Biol. Chem. 253, 4310-4318
27. 27 York, C. M., Cantrell, C. R. and Borum, P. R (1983) Cardiac carnitine deficiency and altered carnitine transport in cardiomyopathic hamsters. Arch. Biochem. Biophys. 221, 526-533
28. 28 Whitmer, J. T. (1986) Energy metabolism and mechanical function in perfused hearts of Syrian hamsters with dilated or hypertrophic cardiomyopathy. J. Mol. Cell. Cardiol. 18, 307-317
29. 29 Hoppel, C. L., Tandler, B., Parland, W., Turkaly, J. S. and Alberts, L. D. (1982) Hamster cardiomyopathy. A defect in oxidative phosphorylation in the cardiac interfibrillar mitochondria. J. Biol. Chem. 257, 1540-1548
30. 30 Nienaber, C. A., Gambhir, S. S., Mody, F. V. et al. (1993) Regional myocardial blood flow and glucose utilization in symptomatic patients with hypertrophic cardiomyopathy. Circulation 87, 1580-1590
31. 31 Grover-McKay, M., Schwaiger, M., Krivokapich, J., Perloff, J. K., Phelps, M. E. and Schelben, H. R. (1989) Regional myocardial blood flow and metabolism at rest in mildly symptomatic patients with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 13, 317-324
32. 32 Rebouche, C. J. and Engel, A. G. (1981) Primary systemic carnitine deficiency. Neurology 31, 813-818
33. 33 Ino, T., Sherwood, W. G., Benson, L. N., Wilson, G. J., Freedom, R. M. and Rowe, R. D. (1988) Cardiac manifestations in disorders of fat and carnitine metabolism in infancy. J. Am. Coll. Cardiol. 11, 1301-1308
34. 34 Kudoh, T., Tamaki, N., Magata, Y. et al. (1997) Metabolism substrate with negative myocardial uptake of iodine-123-BMIPP. J. Nucl. Med. 38, 548-553
35. 35 Fujibayashi, Y., Nohara, R., Hosokawa, R. et al. (1996) Metabolism and kinetics of iodine-123-BMIPP in canine myocardium. J. Nucl. Med. 37, 757-761
36. 36 Hosokawa, R., Nohara, R., Fujibayashi, Y. et al. (1996) Metabolic fate of iodine-123-BMIPP in canine myocardium after administration of etomoxir. J. Nucl. Med. 37, 1836-1840
37. 37 Fujibayashi, Y., Yonekura, Y., Takemura, Y. et al. (1990) Myocardial accumulation of iodinated beta-methyl-branched fatty acid analogue, iodine-125-15-(piodophenyl)-3-(R,S)methylpentadecanoic acid (BMIPP), in relation to ATP concentration. J. Nucl. Med. 31, 1818-1822
38. 38 Kurata, C., Wakabayashi, S., Shouda, S. et al. (1997) Influence of blood substrate levels on myocardial kinetics of iodine-123-BMIPP. J. Nucl. Med. 38, 1079-1084
39. 39 Kuribayashi, T. and Roberts, W. C. (1992) Myocardial disarray at junction of ventricular septum and left and right ventricular free walls in hypertrophic cardiomyopathy. Am. J. Cardiol. 70, 1333-1340
40. 40 Maron, B. J., Ferrans, V.J., Henry, W. L. et al. (1974) Differences in distribution of myocardial abnormalities in patients with obstructive and nonobstructive asymmetric septal hypertrophy (ASH). Light and electron microscopic findings. Circulation 50, 436-446
41. 41 Kuribayashi, T. (1987) Spontaneously occurring hypertrophic cardiomyopathy in the rat. I. Pathologic features. Jpn. Circ. J. 51, 573-588
42. 42 Ohtsuki, K. (1996) Impairment of myocardial fatty acid metabolism in patients with hypertrophic cardiomyopathy. J. Kyoto Prefect. Univ. Med. 105, 561-580
43. 43 Bahl, J., Navin, T., Manian, A. A. and Bressler, R. (1981) Carnitine transport in isolated adult rat heart myocytes and the effect of 7,8-diOHchlorpromazine. Circ. Res. 48, 378-385