PLEKHM2 and cardiomyopathy: Moreover, our results revealed that impaired mitophagy and elevated ROS levels played important roles in the disease progression of PLEKHM2-deficient cardiomyopathy, and PLEKHM2-WT overexpression restored autophagic flux and improved mitochondrial function, ultimately rescued the disease phenotype of PLEKHM2 deficient cardiomyopathy.