Target type: molecularfunction
Catalysis of the reaction: O2 + progesterone + reduced [NADPH--hemoprotein reductase] = 21-hydroxyprogesterone + H+ + H2O + oxidized [NADPH--hemoprotein reductase]. [PMID:25855791, RHEA:50304]
Progesterone 21-hydroxylase activity is a crucial enzymatic function in the biosynthesis of steroid hormones, specifically the synthesis of cortisol and aldosterone. The enzyme, encoded by the CYP21A2 gene, catalyzes the hydroxylation of progesterone at the carbon-21 position, introducing a hydroxyl group to form 11-deoxycortisol. This reaction is the rate-limiting step in cortisol synthesis and is essential for maintaining normal stress response, blood pressure regulation, and electrolyte balance. Progesterone 21-hydroxylase activity also participates in the synthesis of aldosterone, the primary mineralocorticoid hormone, which regulates sodium and potassium balance in the body. The enzyme utilizes molecular oxygen and NADPH as cofactors to perform the hydroxylation reaction, which involves the transfer of a hydroxyl group from a water molecule to the target carbon atom. The activity is regulated by a complex interplay of factors, including hormones, transcription factors, and enzymatic cofactors. Defects in progesterone 21-hydroxylase activity can lead to various genetic disorders, such as congenital adrenal hyperplasia, characterized by cortisol deficiency and excessive production of androgens.'
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Protein | Definition | Taxonomy |
---|---|---|
Steroid 21-hydroxylase | A steroid 21-hydroxylase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P08686] | Homo sapiens (human) |
Steroid 21-hydroxylase | A steroid 21-hydroxylase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P08686] | Homo sapiens (human) |
Compound | Definition | Classes | Roles |
---|---|---|---|
ketoconazole | 1-acetyl-4-(4-{[2-(2,4-dichlorophenyl)-2-(1H-imidazol-1-ylmethyl)-1,3-dioxolan-4-yl]methoxy}phenyl)piperazine : A dioxolane that is 1,3-dioxolane which is substituted at positions 2, 2, and 4 by imidazol-1-ylmethyl, 2,4-dichlorophenyl, and [para-(4-acetylpiperazin-1-yl)phenoxy]methyl groups, respectively. | dichlorobenzene; dioxolane; ether; imidazoles; N-acylpiperazine; N-arylpiperazine | |
abiraterone | 3beta-hydroxy-Delta(5)-steroid; 3beta-sterol; pyridines | antineoplastic agent; EC 1.14.99.9 (steroid 17alpha-monooxygenase) inhibitor | |
17-(3-pyridyl)androsta-5,16-dien-3-one | 17-(3-pyridyl)androsta-5,16-dien-3-one: a steroidal inhibitor of cytochrome P450(17alpha)/C17-20 lyase; structure given in first source | ||
orteronel | orteronel: non-steroidal 17,20-lyase inhibitor; structure in first source | ||
3beta-hydroxy-17-(1h-benzimidazole-1-yl)androsta-5,16-diene | 3-hydroxy-17-(1H-benzimidazole-1-yl)androsta-5,16-diene: has antineoplastic activity; structure in first source | 3-hydroxy steroid | androgen |