TIMM22 is an essential core component of the TIM22 complex, a voltage-gated translocase that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. The protein functions as part of a twin-pore translocase utilizing membrane potential as the driving force for protein translocation 1. TIMM22 operates alongside other TIM complex components including TIMM23, TIMM17A/B, and TIMM44 to facilitate mitochondrial protein biogenesis 2. Clinically, TIMM22 dysfunction has been implicated in mitochondrial pathology and neurodegeneration. TIMM22 knockdown reduces mitochondrial localization of organic cation transporter 3 (OCT3), thereby decreasing MPP+ accumulation in mitochondria and preventing MPP+-induced neuronal dysfunction, including complex I inhibition and apoptosis 3. Additionally, TIMM22 expression is significantly downregulated in heart failure patients, correlating with impaired mitochondrial quality control and energy production defects 2. TIMM22 also interacts with adenine nucleotide translocase 3 and Tomm20 to regulate PINK1 import and mitophagy in multiple myeloma progression 4. Mutations affecting TIM22 complex assembly, including TIMM22 dysfunction, contribute to Combined Oxidative Phosphorylation Deficiency 43 and related mitochondrial myopathies affecting skeletal muscle and cardiac function.