Journal
BEST PRACTICE & RESEARCH CLINICAL ENDOCRINOLOGY & METABOLISM
Volume 26, Issue 6, Pages 771-790Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.beem.2012.05.002
Keywords
cholesterol transport; steroidogenic acute regulatory protein; translocaton protein; StAR-related lipid transfer (START)-domain \; proteins; cytochrome P450 side chain cleavage; steroid biosynthesis; gonads; adrenals; placenta; brain
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Adrenal gonadal, placental and brain mitochondria contain several steroidogenic enzymes, notably the cholesterol side chain cleavage enzyme, P450scc, which is the enzymatic rate-limiting step in steroidogenesis which determines cellular steroidogenic capacity. Even before this step, the access of cholesterol to this enzyme system is both rate-limiting and the site of acute regulation via the steroidogenic acute regulatory protein (StAR) which interacts with a complex multi-component 'transduceosome' on the outer mitochondria! membrane (OMM). The components of the transduceosome include the 18 kDa translocator protein (TSPO), the voltage-dependent anion channel (VDAC-1), TSPO-associated protein 7 (PAP7, ACBD3 for acyl-CoA-binding-domain 3), and protein kinase A regulatory subunit 1 alpha (PKAR1A). The precise fashion in which these,proteins interact and move cholesterol from the OMM to P450scc, and the means by which cholesterol is loaded into the OMM, remain unclear. Human deficiency diseases have been described for StAR and for P450scc. Mitochondria also contain several 'downstream' steroidogenic enzymes. (C) 2012 Elsevier Ltd. All rights reserved.
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