CD36 Deficiency has Little Influence on the Pathophysiology of Hypertrophic Cardiomyopathy

doi:10.1006/jmcc.1999.0957

Journal of Molecular and Cellular Cardiology

Volume 31, Issue 6, June 1999, Pages 1253-1259

https://doi.org/10.1006/jmcc.1999.0957 Get rights and content

Abstract

CD36 is homologous with myocardial long-chain fatty acid (LCFA) binding protein and has been suggested to relate to myocardial fatty acid metabolism. Myocardial scintigraphy with iodine-123 15- (p -iodophenyl)-3-(R, S)-methylpentadecanoic acid (BMIPP) revealed an impairment in LCFA metabolism chiefly in the hypertrophic myocardium in hypertrophic cardiomyopathy (HCM). Recently, the incidence of CD36 deficiency has been reported to be high in HCM patients, and CD36 deficiency was proposed as an etiology of hereditary HCM. However, the pathophysiological effect of CD36 deficiency on HCM has not been fully investigated. We analysed the expression of CD36 antigens on both platelets and monocytes obtained from 82 patients with HCM using two-color flow cytometry. Among the study patients, seven patients (8.5%) demonstrated type II CD36 deficiency, whereas type I CD36 deficiency was not detected. Two of 23 patients (8.7%) with a family history of HCM and five of 59 patients (8.5%) without a family history of HCM showed type II CD36 deficiency respectively. Contrary to the previous report, three of 53 patients with asymmetric septal hypertrophy (ASH) (5.7%) and four of 29 patients without ASH (13.8%) showed CD36 deficiency. Moreover, clinical characteristics, scintigraphic findings, echocardiographic data, and hemodynamic findings disclosed no significant differences between the HCM patients showing normal CD36 expression and those with CD36 deficiency. The incidence of CD36 deficiency in HCM patients is not higher than in the general population. Therefore, CD36 deficiency is not a characteristic factor of HCM and has little influence on the pathyphysiology of HCM.

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Cited by (16)

Treatment with medium chain fatty acids milk of CD36-deficient preschool children
2018, Nutrition
Citation Excerpt :
Low insulin sensitivity and postprandial hypertriglyceridemia have been documented as metabolic manifestations of CD36 deficiency in adulthood [10–13]. Some adult patients with CD36 deficiency may be susceptible to myocardial damage and cardiomyopathy when exposed to severe infections or massive steroid therapy [14,15]. As for CD36 deficiency in childhood, however, its clinical picture and metabolic effects remain largely unknown [16,17].
CD36 deficiency is characterized by limited cellular long chain fatty acid uptake in the skeletal and cardiac muscles and often causes energy crisis in these muscles. However, suitable treatment for CD36 deficiency remains to be established. The aim of this study was to evaluate the clinical and metabolic effects of medium chain triacylglycerols (MCTs) in two CD36-deficient preschool children who often developed fasting hypoglycemia and exercise-induced myalgia.
Fasting blood glucose, total ketone bodies, and free fatty acids were examined and compared for usual supper diets and for diets with replacement of one component with 2 g/kg of 9% MCT–containing milk (MCT milk). Changes in serum creatine kinase and alanine aminotransferase levels, resulting from replacement of glucose water intake with 1 g/kg of MCT milk and determined by using bicycle pedaling tasks, were examined and compared. Hypoglycemic and/or myalgia episodes in daily life were also investigated.
Biochemically, participants' blood glucose and total ketone bodies levels after overnight fasting substantially increased after dietary suppers containing MCT milk. Increases in serum creatine kinase and alanine aminotransferase levels resulting from the bicycle pedaling task were suppressed by MCT milk. Hypoglycemia leading to unconsciousness and tachycardia before breakfast decreased after introduction of dietary suppers containing MCT milk. Occurrence of myalgia in the lower limbs also decreased after intakes of MCT milk before long and/or strenuous exercising.
Our results suggest that MCTs can prevent fasting hypoglycemia and exercise-induced myalgia in CD36-deficient young children.
Genomic heterogeneity of type II CD36 deficiency
2002, Clinica Chimica Acta
Background: CD36 deficiency has been classified in two types, i.e., type I and type II CD36 deficiency. Possible pathological involvement of CD36 deficiency has been suggested in humans, but is still confounding. Homozygous or compound heterozygous mutations (CD36^−/−) were demonstrated in type I CD36 deficiency, while the genomic or molecular background of type II CD36 deficiency is still unclear, which may bring confounding interpretations of the cause-and-effect events in human CD36 deficiency. In this study, we analyzed the genotype and frequency of type II CD36 deficiency in Japanese populations, and its hereditary pattern in three families. Methods: Genotypes and protein expression levels of CD36 were examined in 238 Japanese subjects. Genotype was analyzed in the coding region of the CD36 gene. The expression level of CD36 protein was analyzed by flow cytometry after staining with monoclonal anti-CD36 antibody and assessed as mean fluorescence intensity (MFI). Results: Among 238 subjects, subjects for wild-type gene (WT), a single mutation (CD36^+/−), and CD36^−/− were 141, 44 and 53, respectively. Monocyte MFI (mean±SD) in subjects for WT, CD36^+/−, and CD36^−/− were 35.7±8.5, 15.2±3.4, and 0.4±0.3, respectively (P<0.0001, between groups). Those of platelets in subjects for WT, CD36^+/−, and CD36^−/− were 27.1±10.6, 11.5±6.3, and 0.5±0.3, respectively (P<0.0001, between groups). Subjects of both WT and CD36^+/− were observed in type II CD36 deficiency. Monocyte and platelet MFI in family members of type II CD36 deficiency and 218 unrelated Japanese suggested that the expression level of CD36 protein in monocytes was directly dependent on genotypes. On the other hand, those in platelets were affected by additional heritable factor(s) in addition to the coding region genotype. Conclusions: MFI in monocytes showed a strong gene-dosage-dependency. On the other hand, MFI in platelets was affected by heritable factor(s) in addition to the coding region genotype, which resulted in heterogeneity of type II CD36 deficiency.
Cardiac fatty acid uptake and transport in health and disease
2000, Cardiovascular Research
CD36 deficiency inhibits proliferation by cell cycle control in skeletal muscle cells
2022, Frontiers in Physiology
Changes in Myocardial Metabolism Preceding Sudden Cardiac Death
2020, Frontiers in Physiology
Membrane fatty acid transporters as regulators of lipid metabolism: Implications for metabolic disease
2010, Physiological Reviews

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^f1: Please address all correspondence to: Tomoki Nakamura, MD, Second Department of Medicine, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-Ku, Kyoto 602-0841, Japan.

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Regular ArticleCD36 Deficiency has Little Influence on the Pathophysiology of Hypertrophic Cardiomyopathy

Abstract

Regular Article
CD36 Deficiency has Little Influence on the Pathophysiology of Hypertrophic Cardiomyopathy