Steroid 11-beta-Hydroxylase (11 beta-Hydroxylase)

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).

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Networked: 270 relevant articles (7 outcomes, 26 trials/studies)

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Bio-Agent Context: Research Results

Experts

1.	Hartmann, Rolf W: 8 articles (01/2015 - 08/2010)
2.	Krone, Nils: 5 articles (10/2013 - 06/2005)
3.	Fraser, Robert: 5 articles (09/2013 - 02/2005)
4.	Davies, Eleanor: 5 articles (09/2013 - 02/2005)
5.	Morel, Y: 5 articles (01/2010 - 11/2000)
6.	Mulatero, Paolo: 4 articles (08/2015 - 07/2002)
7.	Veglio, Franco: 4 articles (08/2015 - 07/2002)
8.	Hu, Qingzhong: 4 articles (09/2014 - 08/2010)
9.	Riepe, Felix G: 4 articles (02/2012 - 06/2005)
10.	Gordon, R D: 4 articles (04/2008 - 11/2000)

Related Diseases

1.	Hypertension (High Blood Pressure) 10/01/2010 - "The associations between CYPB11B2/CYP11B1 polymorphisms and persistent postoperative hypertension were assessed by multivariate analysis. " 12/01/2006 - "No variants were identified in the coding region of CYP11B1 that could account for hypertension and/or a raised ARR. " 12/01/2006 - "We therefore aimed to determine whether there is an association between CYP11B1 variants and hypertension and/or an altered ARR. " 10/01/2003 - "Anaesthesia for a girl with severe hypertension due to 11 beta-hydroxylase deficiency." 11/01/2000 - "Genetic analysis showed two new base substitutions of CYP11B1, a conservative transition at the last base of exon 5, and a IVS8+4A-->G transition in intron 8. Difficulties with suppressive therapy resulted in severe hypertension. "
2.	Cushing Syndrome 12/12/2013 - "Inhibition of CYP11B1 is being evaluated in the clinic for potential treatment of hypercortisol diseases such as Cushing's syndrome. " 07/26/2012 - "CYP11B1 inhibition is a promising therapy for Cushing's syndrome. " 07/26/2012 - "Novel imidazol-1-ylmethyl substituted 1,2,5,6-tetrahydropyrrolo[3,2,1-ij]quinolin-4-ones as potent and selective CYP11B1 inhibitors for the treatment of Cushing's syndrome." 08/08/2013 - "Potent and selective CYP11B1 inhibitors could be promising therapeutics for the treatment of Cushing's syndrome. " 01/01/1990 - "2) Adrenal adenoma formation in patients with Cushing's syndrome is accompanied by a relative blockade of 11 beta-hydroxylase activity. "
3.	Congenital Adrenal Hyperplasia (Hyperplasia, Congenital Adrenal) 08/01/1996 - "This case illustrates difficulties in correct diagnosis, choice of appropriate steroid regimen and monitoring efficacy of treatment in congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. " 11/10/2012 - "The objective of this study was to screen the CYP11B1 gene for mutations in one Vietnamese male suffering from congenital adrenal hyperplasia. " 04/01/1985 - "Studies in three families (A, B, and C) revealed five patients with congenital adrenal hyperplasia (CAH) due to partial and combined 21- and 11 beta-hydroxylase deficiency. " 01/01/1982 - "Studies in 18 Jewish families from Morocco, Tunis, Turkey and Iran revealed 26 patients with congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. " 09/01/1980 - "These findings are consistent with previous in vitro studies and studies in patients with congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency, suggesting that a single enzyme system is responsible for both 11 beta- and 18-hydroxylation of DOC in the adrenal zona fasciculata. "
4.	Virilism (Virilization) 01/01/1989 - "Nevertheless the neonate also has severe virilization of the external genitalia and postnatal hormonal studies confirmed the diagnosis of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. " 03/01/2001 - "Based on the author's experience, prenatal diagnosis and treatment of 21-hydroxylase deficiency is safe and effective in significantly reducing or eliminating virilization in the affected female, and the same outcome seems to be true in the treatment of 11 beta-hydroxylase deficiency. " 01/01/2008 - "A homozygous L299P mutation in the CYP11B1 gene leads to complete virilization in 46,XX individuals with 11-beta-hydroxylase deficiency." 01/01/2007 - "11 beta-Hydroxylase deficiency is a rare form of congenital adrenal hyperplasia, resulting in virilization, glucocorticoid deficiency and hypertension. " 01/01/2001 - "Molecular defects of the CYP11B1 gene lead to 11-hydroxylase deficiency, which is clinically expressed with virilization of the external genitalia of the female and precocious puberty in the male, as well as hypertension in both sexes. "
5.	Necrosis 12/14/1987 - "In addition, metyrapone, an efficient and specific inhibitor of the mitochondrial 11 beta-hydroxylase, provided protection against DMBA-induced toxicity, which is in agreement with previous observations that adrenal necrosis caused by DMBA apparently originates in mitochondria. "

Related Drugs and Biologics

1.	Metyrapone
2.	Aldosterone Synthase (CYP11B2)
3.	Steroid 21-Hydroxylase (21 Hydroxylase)
4.	Aromatase (CYP19)
5.	9,10-Dimethyl-1,2-benzanthracene (DMBA)
6.	Steroid 17-alpha-Hydroxylase (17 alpha Hydroxylase)
7.	Enzymes
8.	Hydrocortisone (Cortisol)
9.	Steroid 11-beta-Hydroxylase (11 beta-Hydroxylase)
10.	Dexamethasone (Maxidex)

Related Therapies and Procedures

1.	Adrenalectomy
2.	Cardiopulmonary Resuscitation (CPR)
3.	Therapeutics