Characterization of altered myocardial fatty acid metabolism in patients with inherited cardiomyopathy

Journal of Inherited Metabolic Disease

Volumn 24, Issue 6, 2001, Pages 657-674

  Bergmann, S R ^a   Herrero, P ^b   Sciacca, R ^a   Hartman, J J ^b   Rubin, P J ^b   Hickey, K T ^a   Epstein, S ^a   Kelly, D P ^b  

^a Columbia University New York ^*  (United States)

^b WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; ACYL COENZYME A DEHYDROGENASE; CARNITINE; LONG CHAIN FATTY ACID; PALMITIC ACID; TRIACYLGLYCEROL;

ADOLESCENT; ADULT; ARTICLE; CARDIOMYOPATHY; CARNITINE DEFICIENCY; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; CYTOSOL; ENZYME DEFICIENCY; ENZYME SUBSTRATE; ESTERIFICATION; EVALUATION; EXTRACTION; FATTY ACID METABOLISM; FATTY ACID OXIDATION; FEMALE; GENETIC DISORDER; HEART MUSCLE METABOLISM; HEART MUSCLE OXYGEN CONSUMPTION; HEART PERFUSION; HEMODYNAMICS; HUMAN; INHERITANCE; INTERSTITIUM; MALE; MATHEMATICAL MODEL; METABOLIC DISORDER; NON INVASIVE MEASUREMENT; PHENOTYPE; POSITRON EMISSION TOMOGRAPHY; SIBLING; SUDDEN DEATH; TURNOVER TIME;

ACETIC ACIDS; ADOLESCENT; ADULT; CARDIOMYOPATHIES; CARNITINE; CHILD; CHILD, PRESCHOOL; CORONARY CIRCULATION; DEATH, SUDDEN; FATTY ACIDS; FEMALE; HEMODYNAMIC PROCESSES; HUMANS; MALE; MYOCARDIUM; OXIDATION-REDUCTION; OXYGEN CONSUMPTION; PALMITIC ACID; PHENOTYPE; TOMOGRAPHY, EMISSION-COMPUTED;

EID: 0035198533     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1012711009687     Document Type: Article

References (30)

1. Long-chain acyl-coenzyme A dehydrogenase deficiency: Biochemical studies in fibroblasts from three patients
2. Noninvasive quantitation of myocardial blood flow in human subjects with oxygen-15-labeled water and positron emission tomography
3. Quantitation of myocardial fatty acid metabolism using PET
4. Delineation of myocardial oxygen utilization with carbon-11 labeled acetate
5. Noninvasive assessment of canine myocardial oxidative metabolism with carbon-11 acetate and positron emission tomography
6. Validity of estimates of myocardial oxidative metabolism with carbon-11 acetate and positron emission tomography despite altered patterns of substrate utilization
7. Effect of carbon-11-acetate recirculation on estimates of myocardial oxygen consumption by PET
8. Coronary hemodynamics and myocardial metabolism during and after pacing stress in normal humans
9. Myocardial metabolism in ischemic heart disease: Basic principles and application to imaging by positron emission tomography
10. Selected metabolic alterations in the ischemic heart and their contributions to arrhythmogenesis
11. Sudden child death and "healthy" affected family members with medium-chain acyl-coenzyme A dehydrogenase deficiency
12. Efflux of metabolized and nonmetabolized fatty acid from canine myocardium. Implications for quantifying myocardial metabolism tomographically
13. Fluorometric assay of acyl-CoA dehydrogenases in normal and mutant human fibroblasts
14. Measurement of myocardial oxygen consumption using positron emission tomography and C-11 acetate: Direct validation in human subjects
15. Fatty acid oxidation disorders: A new class of metabolic diseases
16. Long-chain acyl coenzyme A dehydrogenase deficiency: An inherited cause of nonketotic hypoglycemia
17. 15O and positron emission tomography: Assessment and error analysis of a mathematical approach
18. Effects of time discrepancies between input and myocardial time-activity curves on estimates of regional myocardial perfusion with PET
19. Use of compartmental models of carbon-11 acetate to measure myocardial oxygen consumption: Validation in human subjects
20. Functional correction of short-chain acyl-CoA dehydrogenase deficiency in transgenic mice: Implications for gene therapy of human mitochondrial enzyme deficiencies
21. Inherited cardiomyopathies
22. Detection and assessment by positron emission tomography of a genetically determined defect in myocardial fatty acid utilization (long-chain acyl-CoA dehydrogenase deficiency)
23. Intermittent nonketotic dicarboxylic aciduria in two siblings with hypoglycemia: An apparent defect in β-oxidation of fatty acids
24. 14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts
25. Acyl-CoA dehydrogenase deficiencies
26. Metabolic fate of radiolabeled palmitate in ischemic canine myocardium: Implications for positron emission tomography
27. Recognition and management of fatty acid oxidation defects: A series of 107 patients
28. Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency
29. Disorders of mitochondrial fatty acyl-CoA β-oxidation
30. Remote system for production of carbon-11 labeled palmitic acid