Meanwhile, hyper-methylation of mitochondrial ribosomes proved to be a critical molecular defect driving the apoptotic phenotype and deafness in Tg-B1 mice induced by a pro-apoptotic, ROS-AMPK-E2F1 pathway [14], which manifested as increased E2F1 and apoptosis in the Stria Vascularis (SV) and SGNs of the inner ear, and progressive E2F1-dependent hearing loss. The gene discussed is PRKAA2; the disease is deafness.