1.14.15.4: steroid 11beta-monooxygenase
This is an abbreviated version!
For detailed information about steroid 11beta-monooxygenase, go to the full flat file.
Word Map on EC 1.14.15.4
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1.14.15.4
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hypertension
-
cortisol
-
adrenocortical
-
steroidogenic
-
corticosterone
-
angiotensin
-
hyperplasia
-
acth
-
zona
-
glucocorticoid
-
steroidogenesis
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mineralocorticoid
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glomerulosa
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adenoma
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21-hydroxylase
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renin
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11-deoxycortisol
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deoxycorticosterone
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fasciculata
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metyrapone
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11-deoxycorticosterone
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beta-hydroxysteroid
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pregnenolone
-
angiotensinogen
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cushing
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cyp11a1
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virilization
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androstenedione
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cyp21
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spironolactone
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18-hydroxycorticosterone
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p450scc
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adrenodoxin
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17-hydroxyprogesterone
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hirsutism
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beta-hydroxylation
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atp1a1
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acth-stimulated
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17alpha-hydroxylase
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cortisol-producing
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etomidate
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hsd3b2
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hypokalemia
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medicine
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trilostane
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apccs
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salt-wasting
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11-ketotestosterone
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low-renin
-
drug development
-
liddle
-
extra-adrenal
-
analysis
- 1.14.15.4
- hypertension
- cortisol
-
adrenocortical
-
steroidogenic
- corticosterone
- angiotensin
- hyperplasia
- acth
- zona
- glucocorticoid
-
steroidogenesis
-
mineralocorticoid
- glomerulosa
- adenoma
- 21-hydroxylase
- renin
- 11-deoxycortisol
- deoxycorticosterone
- fasciculata
- metyrapone
- 11-deoxycorticosterone
-
beta-hydroxysteroid
- pregnenolone
- angiotensinogen
- cushing
- cyp11a1
-
virilization
- androstenedione
- cyp21
- spironolactone
- 18-hydroxycorticosterone
- p450scc
- adrenodoxin
- 17-hydroxyprogesterone
- hirsutism
-
beta-hydroxylation
- atp1a1
-
acth-stimulated
- 17alpha-hydroxylase
-
cortisol-producing
- etomidate
- hsd3b2
- hypokalemia
- medicine
- trilostane
-
apccs
-
salt-wasting
- 11-ketotestosterone
-
low-renin
- drug development
-
liddle
-
extra-adrenal
- analysis
Reaction
Synonyms
11-hydroxylase, 11 beta-hydroxylase, 11-beta hydroxylase, 11-beta-hydroxylase, 11-hydroxylase, 11beta hydroxylase, 11beta-hydroxylase, ALDOS, Aldosterone synthase, Aldosterone-synthesizing enzyme, C450XIB2, CYP11B1, CYP11B2, cyp11beta, CYPXIB, CYPXIB1, CYPXIB2, CYPXIB3, cytochrome 11beta-hydroxylase, cytochrome P-45011-beta, cytochrome P450 11B1, cytochrome P450 11B2, cytochrome P450 11beta-hydroxylase, cytochrome P45011beta, cytochrome P45011beta-hydroxylase, EC 1.14.1.6, EC 1.99.1.7, P-450(11 beta,aldo), P-450Aldo, P-450c11, P-450C18, P-450XIB1, P450 11beta-hydroxylase, P450(11 beta)-DS, P45011beta, P450C11, Steroid 11-beta-hydroxylase, steroid 11-hydroxylase, steroid 11beta-hydroxylase, steroid 11beta-monooxygenase, steroid 11beta/18-hydroxylase, Steroid 18-hydroxylase, steroid-11beta-hydroxylase
ECTree
1.14.15.4 steroid 11beta-monooxygenaseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 1.14.15.4 - steroid 11beta-monooxygenase
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
11-deoxy-cortisol + reduced ferredoxin + O2
cortisol + ferredoxin + H2O
-
-
-
-
?
11-deoxycorticosterone + reduced adrenal ferredoxin + O2
corticosterone + oxidized adrenal ferredoxin + H2O
-
11beta-hydroxylation, last 3 steps of pathway for aldosterone biosynthesis
-
?
11-deoxycorticosterone + reduced adrenodoxin + O2
corticosterone + oxidized adrenodoxin + H2O
11-deoxycortisol + adrenal ferredoxin + H2O
cortisol + reduced adrenal ferredoxin + O2
-
-
-
-
?
11-deoxycortisol + reduced adrenal ferredoxin + O2
cortisol + oxidized adrenal ferredoxin + H2O
16,17alpha-epoxyprogesterone + reduced adrenal ferredoxin + O2
11beta-hydroxy-16,17alpha-epoxyprogesterone + adrenal ferredoxin + H2O
18-hydroxy-corticosterone + reduced adrenal ferredoxin + O2
aldosterone + oxidized adrenal ferredoxin + H2O
-
18-hydroxylation, last 3 steps of pathway for aldosterone biosynthesis
-
?
18-hydroxycorticosterone + reduced adrenal ferredoxin + O2
aldosterone + oxidized adrenal ferredoxin + H2O
18-hydroxycorticosterone + reduced adrenodoxin + O2
aldosterone + oxidized adrenodoxin + H2O
-
-
-
?
corticosterone + reduced adrenal ferredoxin + O2
11-deoxycorticosterone + adrenal ferredoxin + ?
-
-
-
-
?
corticosterone + reduced adrenal ferredoxin + O2
18-hydroxy-corticosterone + oxidized adrenal ferredoxin + H2O
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18-hydroxylation, last 3 steps of pathway for aldosterone biosynthesis
-
?
corticosterone + reduced adrenodoxin + O2
18-hydroxycorticosterone + oxidized adrenodoxin + H2O
cortisol + reduced adrenal ferredoxin + O2
11-deoxycortisol + adrenal ferredoxin + ?
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in the adrenal zona fasciculata
-
-
?
11-deoxycorticosterone + reduced adrenodoxin + O2
corticosterone + oxidized adrenodoxin + H2O
-
-
-
?
11-deoxycorticosterone + reduced adrenodoxin + O2
corticosterone + oxidized adrenodoxin + H2O
-
-
-
?
11-deoxycortisol + reduced adrenal ferredoxin + O2
cortisol + oxidized adrenal ferredoxin + H2O
-
-
-
-
?
11-deoxycortisol + reduced adrenal ferredoxin + O2
cortisol + oxidized adrenal ferredoxin + H2O
-
-
-
?
11-deoxycortisol + reduced adrenal ferredoxin + O2
cortisol + oxidized adrenal ferredoxin + H2O
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CYP11B1
-
-
?
11-deoxycortisol + reduced adrenal ferredoxin + O2
cortisol + oxidized adrenal ferredoxin + H2O
-
deficiency of steroid 11-hydroxylase CYP11B1 due to mutations in the CYP11B1 gene causes congenital adrenal hyperplasia, a group of autosomal recessive disorders, overview
-
-
?
16,17alpha-epoxyprogesterone + reduced adrenal ferredoxin + O2
11beta-hydroxy-16,17alpha-epoxyprogesterone + adrenal ferredoxin + H2O
-
-
the product is an important intermediate for many anti-inflammatory drugs
-
?
16,17alpha-epoxyprogesterone + reduced adrenal ferredoxin + O2
11beta-hydroxy-16,17alpha-epoxyprogesterone + adrenal ferredoxin + H2O
-
-
the product is an important intermediate for many anti-inflammatory drugs
-
?
18-hydroxycorticosterone + reduced adrenal ferredoxin + O2
aldosterone + oxidized adrenal ferredoxin + H2O
-
-
-
-
?
18-hydroxycorticosterone + reduced adrenal ferredoxin + O2
aldosterone + oxidized adrenal ferredoxin + H2O
-
-
-
?
18-hydroxycorticosterone + reduced adrenal ferredoxin + O2
aldosterone + oxidized adrenal ferredoxin + H2O
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CYP11B2
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-
?
18-hydroxycorticosterone + reduced ferredoxin + O2
aldosterone + ferredoxin + H2O
-
aldosterone is one of the main effectors of the reninangiotensinaldosterone system, RAAS, regulating salt and water homeostasis and thereby also blood pressure
-
-
?
18-hydroxycorticosterone + reduced ferredoxin + O2
aldosterone + ferredoxin + H2O
-
-
-
-
?
corticosterone + reduced adrenodoxin + O2
18-hydroxycorticosterone + oxidized adrenodoxin + H2O
-
-
-
?
corticosterone + reduced adrenodoxin + O2
18-hydroxycorticosterone + oxidized adrenodoxin + H2O
-
-
-
?
additional information
?
-
-
the enzyme is involved in sex differentiation in the protandrous anemonefish, overview
-
-
?
additional information
?
-
-
corticosterone generally believed to be preferred substrate for 18-hydroxylase
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-
?
additional information
?
-
the enzyme is involved in gonadal transformation in the zebrafish, enzyme expression regulation, overview
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-
?
additional information
?
-
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the enzyme is involved in gonadal transformation in the zebrafish, enzyme expression regulation, overview
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-
?
additional information
?
-
-
a key mitochondrial enzyme for the production of 11-oxygenated androgens, which have been shown to be potent masculinising steroids in several fish species, it plays a role in testicular development
-
-
?
additional information
?
-
-
-344T/C polymorphism is associated with a clinically relevant phenotype for diabetic nephropathy such as hypertension
-
-
?
additional information
?
-
-
CYP11B2-CYP11B1 haplotypes associate with decreased 11beta-hydroxylase activity, overview
-
-
?
additional information
?
-
-
effects of enzyme inhibition of steroid biosynthesis and mineralcorticoid formation, overview
-
-
?
additional information
?
-
-
enzyme deficiency is a cause for congenital adrenal hyperplasia, overview
-
-
?
additional information
?
-
-
genetic variation at the locus encompassing 11-beta hydroxylase and aldosterone synthase accounts for heritability in cortisol precursor 11-deoxycortisol urinary metabolite excretion, overview
-
-
?
additional information
?
-
-
non-classical enzyme deficiency causes mild androgen excess, hirsutism, precocious adrenarche and accelerated growth in children, missense mutations are responsible for non-classical adrenal hyperplasia in children, overview
-
-
?
additional information
?
-
-
variation of 344 C/T and intron 2 in the CYP11B2 gene, encoding aldosterone synthase, is associated with CYP11B1 deficiency due to variations of gene CYP11B1 at positions 1889 and 1859 and disequiliibrium with CYP11B2, overview
-
-
?
additional information
?
-
-
11-beta-hydroxylase deficiency, i.e. 11betaOHD is caused by CYP11B1 gene defects and leads to congenital adrenal hyperplasia associated with hypertension, overview
-
-
?
additional information
?
-
-
11beta-hydroxylase deficiency is the second most common form of congenital adrenal hyperplasia after 21-hydroxylase deficiency, overview
-
-
?
additional information
?
-
-
congenital adrenal hyperplasia due to steroid 11beta-hydroxylase deficiency is a genetic disorder of steroidogenesis, transmitted as an autosomal recessive trait. It is associated with low renin hypertension, hypokalemia, hyperandrogenemia and genital ambiguity in affected females, overview
-
-
?
additional information
?
-
-
deficiency of 11beta-hydroxylase results in decreased synthesis of cortisol and glucocorticoids, and thus causes weakened depressive feedback ofACTH and increased synthesis of ACTH in pituitary, which leads to production of more cortisol precursor in zona fasciculata, enzyme regulation, overview
-
-
?
additional information
?
-
-
enzyme deficiency leads to hypokalemia and further, when under-treated, to rhabdomyolysis, overview
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-
?
additional information
?
-
-
the enzyme is regulated by adrenocorticotrophic hormone, ACTH
-
-
?
additional information
?
-
-
the polymorphism genotype of gene CYP11B2, encoding aldosterone synthase, affects the activity of the 11beta-hydroxylase, encoded by gene CYP11B1, overview
-
-
?
additional information
?
-
enzyme is responsible for the final step of aldosterone synthesis. It requires electron transfer from the adrenodoxin/adrenodoxin reductase system to catalyze the production of aldosterone
-
-
?
additional information
?
-
enzyme is responsible for the final step of aldosterone synthesis. It requires electron transfer from the adrenodoxin/adrenodoxin reductase system to catalyze the production of aldosterone
-
-
?
additional information
?
-
-
enzyme is responsible for the final step of aldosterone synthesis. It requires electron transfer from the adrenodoxin/adrenodoxin reductase system to catalyze the production of aldosterone
-
-
?
additional information
?
-
-
CYP11B2 hydroxylates 18-hydroxycorticosterone
-
-
?
additional information
?
-
-
steroid 11beta-hydroxylase converts 11-deoxycortisol and 11-deoxycorticosterone to cortisol and corticosterone, respectively
-
-
?
additional information
?
-
bifunctional enzyme catalyzing the biosynthesis of aldosterone by successive 11beta- and 18-hydroxylation followed by an 18-oxidation of 11-deoxycorticosterone
-
-
?
additional information
?
-
-
bifunctional enzyme catalyzing the biosynthesis of aldosterone by successive 11beta- and 18-hydroxylation followed by an 18-oxidation of 11-deoxycorticosterone
-
-
?
additional information
?
-
-
N-terminally unprocessed enzyme has 2fold more activity than the mature physiological enzyme
-
-
?
additional information
?
-
the concomitant down-regulation of P450arom and up-regulation of P45011beta are of pivotal importance to the sex change
-
-
?
additional information
?
-
-
the concomitant down-regulation of P450arom and up-regulation of P45011beta are of pivotal importance to the sex change
-
-
?
additional information
?
-
-
prolactin regulatory element-binding (PREB) protein. It regulates the transcription of the CYP11B1 gene via cAMP. PREB can function as a transcriptional regulator of the CYP11B1 promoter. PREB binds to the CYP11B1 promoter, and in cells expressing PREB, it increases CYP11B1 expression. Knockdown of PREB expression attenuated the effects of cAMP on CYP11B1 expression. CYP11B1 promoter activity is increased in the cells treated with the scrambled siRNA and cAMP, while the CYP11B1 promoter activity is markedly reduced in the cells treated with the PREB-specific siRNA. Enzyme is regulated by cAMP, which stimulates the expression of PREB. Knockdown of PREB expression attenuates the effects of cAMP on CYP11B1 expression
-
-
?
additional information
?
-
-
responsible for the last step of glucocorticoid biosynthesis in the adrenal cortices of many kinds of animals
-
-
?
additional information
?
-
-
androgen production of male animal is partially regulated by changes in abundance of enzyme mRNA
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-
?
