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Major advances have been made during the last decadein our understanding of adrenal steroid hormone biosynthesis. Two key playersin these pathways are the human mitochondrial cytochrome P450 enzymes CYP11B1and CYP11B2, which catalyze the final steps in the biosynthesis of cortisol andaldosterone. Using data from mutations found in patients suffering from steroidhormonerelated diseases, from mutagenesis studies and from the construction ofthreedimensional models of these enzymes, structural information could bededuced that provide a clue to the stereo and regiospecific steroidhydroxylation reactions carried out by these enzymes. In this review, wesummarize the current knowledge on the physiological function and thebiochemistry of these enzymes. Furthermore, the pharmacological andtoxicological importance of these steroid hydroxylases, the means for theidentification of their potential inhibitors and possible biotechnologicalapplications are discussed.

The Human Steroid Hydroxylases CYP11B1 and CYP11B2