HADHA and hypertrophic cardiomyopathy: In detail, depletion of SIRT5 enhances ECHA Ksuc at K315, which inhibits ECHA enzymatic activity, leading to defective fatty acid metabolism, decreased ATP production, and hypertrophic cardiomyopathy (Fig. 4c).40 In another study, differential protein expression and succinylated lysine residues in myofibrils from failing ischemic cardiomyopathy hearts and non-failing hearts in patients were evaluated.