Cardiomyocyte steatosis and defective washout of iodine-123-β-methyl iodophenyl-pentadecanoic acid in genetic deficiency of adipose triglyceride lipase

Long-chain fatty acid (LCFA) is a major energy source for the heart. LCFA is taken up through CD36 and transported to mitochondria for β-oxidation to produce ATP. Therefore, in contrast to adipocytes where LCFA is stored as triglyceride (TG) in cytoplasmic lipid droplets, cardiomyocytes usually do not have lipid droplets. We previously reported a novel phenotype called ‘Triglyceride deposit cardiomyovasculopathy’ (TGCV) in a cardiac transplant (CTx) recipient (Case 1). TGCV is characterized by the massive accumulation of TG in both myocardium and coronary arteries. Case 1 was …