In pathological conditions such as heart failure and cardiomyopathy, impaired BCAA catabolism, often due to downregulation of Protein phosphatase 2Cm (PP2Cm) or reduced BCKDH activity, can lead to the accumulation of branched-chain keto acids (BCKAs), which inhibit mitochondrial complex I, promote excessive reactive oxygen species (ROS) production, and compromise mitochondrial function. This evidence concerns the gene PPM1K and cardiomyopathy.