Inner Mitochondrial Translocase Tim50 Is Central in Adrenal and Testicular Steroid Synthesis.

ABSTRACT: Adrenal and gonadal mitochondrial metabolic activity requires electrons from cofactors, cholesterol, and a substrate for rapid steroid synthesis, an essential requirement for mammalian survival. Substrate activity depends on its environment, which is regulated by chaperones and mitochondrial translocases. Cytochrome P450 side-chain cleavage enzyme (SCC or CYP11A1) catalyzes cholesterol to pregnenolone conversion, although its mechanism of action is not well understood. We find that SCC is directly imported into the mitochondrial matrix, where its N-terminal sequence is cleaved sequentially, after which it becomes activated following the second cleavage, which is dependent on the folding of the protein. Following integration of the SCC C terminus into the TIM23 complex, amino acids 141 to 1

SUBMITTER: Bose HS 

PROVIDER: S-EPMC6290369 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature