Target type: molecularfunction
Catalysis of the reaction: 17alpha-hydroxyprogesterone + O2 + reduced [NADPH--hemoprotein reductase] = 11-deoxycortisol + H+ + H2O + oxidized [NADPH--hemoprotein reductase]. [GOC:pz, RHEA:50308]
17-hydroxyprogesterone 21-hydroxylase activity is a crucial enzyme involved in the biosynthesis of cortisol and aldosterone, essential hormones for regulating various physiological processes. This enzyme catalyzes the hydroxylation of 17-hydroxyprogesterone at the C21 position, converting it to 11-deoxycortisol. This reaction occurs in the mitochondria of adrenal cortex cells and involves the transfer of an oxygen atom from molecular oxygen to the substrate, requiring the presence of NADPH and molecular oxygen. 17-hydroxyprogesterone 21-hydroxylase deficiency, a genetic disorder, results in impaired cortisol and aldosterone synthesis, leading to various symptoms including salt wasting, hypertension, and hypoglycemia. The enzyme's activity is tightly regulated by various factors, including ACTH stimulation and steroidogenic enzymes expression. This activity is essential for maintaining homeostasis and overall health, and its malfunction can have significant clinical consequences.'
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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) |
| 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 |