CYP11B1; S11BH; Cytochrome P450 11B1, mitochondrial; CYPXIB1; Cytochrome P-450c11; Cytochrome P450C11; Steroid 11-beta-hydroxylase; CYP11B2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

CYP11B1/2 Polyclonal Antibody detects endogenous levels of CYP11B1/2 protein.

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

1 mg/ml (lot specific)

Store at -20 degree C/1 year

Small volumes of anti-CYP11B1/2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Total protein Ab

Western Blot (WB), ELISA (EIA)

Western Blot: 1:500 - 1:2000
ELISA: 1:20000
Not yet tested in other applications.

Western Blot analysis of HT29 HuvEc K562 cells using CYP11B1/2 Polyclonal Antibody

49,750 Da

cytochrome P450, family 11, subfamily B, polypeptide 1

cytochrome P450 11B1, mitochondrial

Cytochrome P450 11B1, mitochondrial

S11BH; Cytochrome P450C11??[Similar Products]

C11B1_HUMAN

This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane and is involved in the conversion of progesterone to cortisol in the adrenal cortex. Mutations in this gene cause congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency. Transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]

CYP11B1: 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. Defects in CYP11B1 are the cause of adrenal hyperplasia type 4 (AH4). AH4 is 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 ***** 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). AH4 patients usually have hypertension. Defects in CYP11B1 are a cause of familial hyperaldosteronism type 1 (FH1). It is 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. The molecular defect causing hyperaldosteronism familial type 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. Belongs to the cytochrome P450 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Oxidoreductase; Lipid Metabolism - C21-steroid hormone; Mitochondrial; Lipid Metabolism - androgen and estrogen; EC 1.14.15.4

Chromosomal Location of Human Ortholog: 8q21

Cellular Component: mitochondrion; mitochondrial inner membrane

Molecular Function: steroid 11-beta-monooxygenase activity; iron ion binding; heme binding

Biological Process: steroid metabolic process; regulation of blood pressure; xenobiotic metabolic process; C21-steroid hormone biosynthetic process; immune response; glucocorticoid biosynthetic process; glucose homeostasis; sterol metabolic process; aldosterone biosynthetic process; cellular response to hormone stimulus

Disease: Adrenal Hyperplasia, Congenital, Due To Steroid 11-beta-hydroxylase Deficiency; Glucocorticoid-remediable Aldosteronism

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