TIM50 Antibody #62317

Mitochondria play a central role in cellular energy metabolism and are essential organelles in eukaryotes. In humans, 13 proteins are encoded by the mitochondrial genome while the vast majority of mitochondrial proteins are encoded by the nuclear genome. As a result, most mitochondrial proteins are synthesized as precursors in the cytoplasm and imported across mitochondrial membranes by one or more translocase protein complexes (1). The translocase of the outer mitochondrial membrane (TOM complex) facilitates the import of proteins through the outer mitochondrial membrane, while the complementary translocase of the inner membrane (TIM23 complex) is responsible for protein transport to the mitochondrial matrix. Tim21 facilitates protein translocation from the outer to the inner membrane through the inter-membrane space. The TIM23 complex is associated with PAM, the motor which transports proteins across the inner membrane and into the matrix. The TIM23 complex is formed by three essential inner membrane proteins: Tim50 (receptor), Tim23 (channel-forming), and Tim17 (motor recruitment and sorting function) (1). 

TIM50, encoded by the TIMM50 gene, is ubiquitously expressed and mutations in this gene cause severe mitochondrial dysfunction as well as compromised metabolism (2). In addition to its receptor function, TIM50 can close the Tim23 channel which is important in order to maintain the electrochemical proton gradient for ATP synthesis while transporting cargo across the membrane (3).