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DrugBank target: P15538

DrugBank target: P15538 (C11B1_HUMAN)     

Cytochrome P450 11B1, mitochondrial

CYPXIB1, Cytochrome P-450c11, Steroid 11-beta-hydroxylase, Cytochrome P450C11.

Function

Has steroid 11-beta-hydroxylase activity. In addition to this activity, the 18 or 19-hydroxylation of steroids and the aromatization of androstendione to estrone have also been ascribed to cytochrome P450 XIB..

Enzyme

1.14.15.4   [EC->PDB]   [IntEnz]   [ExPASy]   [KEGG]  

Catalytic activity

A steroid + 2 reduced adrenodoxin + O(2) + 2 H(+) = an 11-beta- hydroxysteroid + 2 oxidized adrenodoxin + H(2)O.

Disease(s)

Adrenal hyperplasia 4 (AH4) [MIM:202010]: A form of congenital adrenal hyperplasia, a common recessive disease due to defective synthesis of cortisol. Congenital adrenal hyperplasia is characterized by androgen excess leading to ambiguous genitalia in affected females, rapid somatic growth during childhood in both sexes with premature closure of the epiphyses and short adult stature. Four clinical types: 'salt wasting' (SW, the most severe type), 'simple virilizing' (SV, less severely affected patients), with normal aldosterone biosynthesis, 'non-classic form' or late- onset (NC or LOAH)and 'cryptic' (asymptomatic). {ECO:0000269|PubMed:16046588, ECO:0000269|PubMed:2022736, ECO:0000269|PubMed:20331679, ECO:0000269|PubMed:20947076, ECO:0000269|PubMed:9302260}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Familial hyperaldosteronism 1 (FH1) [MIM:103900]: A disorder characterized by hypertension, variable hyperaldosteronism, and abnormal adrenal steroid production, including 18-oxocortisol and 18-hydroxycortisol. There is significant phenotypic heterogeneity, and some individuals never develop hypertension. Note=The disease is caused by mutations affecting the gene represented in this entry. The molecular defect causing hyperaldosteronism familial 1 is an anti-Lepore-type fusion of the CYP11B1 and CYP11B2 genes. The hybrid gene has the promoting part of CYP11B1, ACTH-sensitive, and the coding part of CYP11B2.

Structures

There are currently no structures for this UniProt code in the PDB.

Schematic diagram of Pfam domains in target sequence

Key:    PfamA domain

Sequence length: 503 a.a.

Approved drugs targeting this protein

The DrugBank database identifies 2 drugs for this target protein:

 

Generic name: DB01011 metyrapone
MYT
Formula: C14H14N2O
Structure: There are 2 PDB structures containing this molecule although none are bound to the above target protein.
 
Generic name: DB00648 mitotane
Formula: C14H10Cl4
Structure: There are no structures of this molecule in the PDB.

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