Fig. 3
From: Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder
A pathophysiological model for TGCV. Genetic and acquired ATGL deficiency and other causes result in abnormal intracellular metabolism of TG and LCFA, leading to cardiomyocyte steatosis and TG-deposit SMCs. In normal condition (left panel), LCFA is taken up through LCFA transporters and receptors such as CD36 and some of them are transported to mitochondria for β-oxidation and the remaining LCFAs are utilized as a source for TG and rapidly hydrolyzed by intracellular lipases such as ATGL. In TGCV (right panel), LCFAs are taken up and used for the synthesis of TG which can not be hydrolyzed, leading to massive TG accumulation