Source:http://linkedlifedata.com/resource/umls/id/C0038296
MSH: A mitochondrial cytochrome P450 enzyme that catalyzes the 11-beta-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP11B1 gene, is important in the synthesis of CORTICOSTERONE and HYDROCORTISONE. Defects in CYP11B1 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).,NCI: Encoded by human CYP11B1 Gene (Cytochrome P450 Family), 503-aa 58-kDa (precursor) Cytochrome P450 CYP11B1 is a mitochondrial inner membrane P450 heme-thiolate monooxygenase expressed in adrenal cortex with steroid 11-beta-hydroxylase activity and involved in conversion of progesterone to cortisol. The 18 or 19-hydroxylation of steroids and aromatization of androstendione to estrone have also been ascribed to CYP11B1. CYP11B1 defects cause adrenal hyperplasia type IV (AH-IV). CYP11B1/CYP11B2 gene fusion causes glucocorticoid-remediable aldosteronism (GRA). P450 enzymes catalyze reactions involved in drug metabolism and synthesis of cholesterol,