catalog number :
MBS601305
products full name :
Cytochrome P450 Aldosterone Synthase (CYP11b2)
products short name :
Cytochrome P450 Aldosterone Synthase
products name syn :
Anti -Cytochrome P450 Aldosterone Synthase (CYP11b2)
other names :
cytochrome P450 11B2, mitochondrial; Cytochrome P450 11B2, mitochondrial; cytochrome P450 11B2, mitochondrial; cytochrome P-450C18; aldosterone synthase; cytochrome P-450Aldo; steroid 11-beta-monooxygenase; steroid 11-beta/18-hydroxylase; aldosterone-synthesizing enzyme; steroid 18-hydroxylase, aldosterone synthase, P450C18, P450aldo; mitochondrial cytochrome P450, family 11, subfamily B, polypeptide 2; cytochrome P450, subfamily XIB (steroid 11-beta-hydroxylase), polypeptide 2; cytochrome P450, family 11, subfamily B, polypeptide 2; Aldosterone synthase (EC:1.14.15.4, EC:1.14.15.5); ALDOS; Aldosterone-synthesizing enzyme; CYPXIB2; Cytochrome P-450Aldo; Cytochrome P-450C18; Steroid 18-hydroxylase
other gene names :
CYP11B2; CYP11B2; CPN2; ALDOS; CYP11B; CYP11BL; CYPXIB2; P450C18; P-450C18; P450aldo; ALDOS
uniprot entry name :
C11B2_HUMAN
specificity :
Recognizes rat cytochrome p450 aldosterone synthase. Species Crossreactivity: mouse
purity :
Supernatant. Supernatant
form :
Supplied as a liquid, 50% glycerol, 0.1% sodium azide.
storage stability :
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC)
app notes :
Suitable for use in Western Blot, Immunohistochemistry, Immunocytochemistry and ELISA. Dilution: Western Blot: A band is observed at ~48kD in adrenal zona glomerulosa mitochondrial preparations. The enzyme is expressed best in adrenal zona glomerulosa tissues, expression in kidney is virtually undetectable. Immunohistochemistry: 1:50-1:200 (ABC, enzymatic detection). Works well with STF fixed tissues that are paraffin embedded. Also works with formalin fixed paraffin embedded tissues but at much lower working dilutions. Recommended counterstain is Gill's Hematoxylin. Immunocytochemistry: 1:20-1:200 on acetone fixed cells. Optimal dilutions to be determined by researcher.
other info1 :
Immunogen: Rat Aldo-1-MAP aa175-192, KVRQNARGSLTMDVQQSL-MAP.
products categories :
Antibodies; Abs to Enzymes, Cytochrome
products description :
Aldosterone synthase (or 18-hydroxylase) is a steroid hydroxylase cytochrome P450 oxidase enzyme involved in the generation of aldosterone. 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. The enzyme has steroid 18-hydroxylase activity to synthesize aldosterone and 18-oxocortisol as well as steroid 11 beta-hydroxylase activity.
ncbi acc num :
NP_000489.3
ncbi gb acc num :
NM_000498.3
ncbi mol weight :
57,560 Da
ncbi pathways :
ACE Inhibitor Pathway (198763); Biological Oxidations Pathway (105698); C21-Steroid Hormone Biosynthesis, Progesterone = Corticosterone/aldosterone Pathway (413394); C21-Steroid Hormone Biosynthesis, Progesterone = Corticosterone/aldosterone Pathway (468295); C21-Steroid Hormone Biosynthesis, Progesterone = Cortisol/cortisone Pathway (413395); C21-Steroid Hormone Biosynthesis, Progesterone = Cortisol/cortisone Pathway (468370); Cytochrome P450 - Arranged By Substrate Type Pathway (105700); Endogenous Sterols Pathway (105701); Glucocorticoid Mineralcorticoid Metabolism Pathway (198902); Glucocorticoid Biosynthesis Pathway (106152)
ncbi summary :
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. The enzyme has steroid 18-hydroxylase activity to synthesize aldosterone and 18-oxocortisol as well as steroid 11 beta-hydroxylase activity. Mutations in this gene cause corticosterone methyl oxidase deficiency. [provided by RefSeq, Jul 2008]
uniprot summary :
CYP11B2: Preferentially catalyzes the conversion of 11- deoxycorticosterone to aldosterone via corticosterone and 18- hydroxycorticosterone. Defects in CYP11B2 are the cause of corticosterone methyloxidase type 1 deficiency (CMO-1 deficiency); also known as aldosterone deficiency due to defect in 18- hydroxylase or aldosterone deficiency I. CMO-1 deficiency is an autosomal recessive disorder of aldosterone biosynthesis. There are two biochemically different forms of selective aldosterone deficiency be termed corticosterone methyloxidase (CMO) deficiency type 1 and type 2. In CMO-1 deficiency, aldosterone is undetectable in plasma, while its immediate precursor, 18- hydroxycorticosterone, is low or normal. Defects in CYP11B2 are the cause of corticosterone methyloxidase type 2 deficiency (CMO-2 deficiency). CMO-2 is an autosomal recessive disorder of aldosterone biosynthesis. In CMO-2 deficiency, aldosterone can be low or normal, but at the expense of increased secretion of 18- hydroxycorticosterone. Consequently, patients have a greatly increased ratio of 18-hydroxycorticosterone to aldosterone and a low ratio of corticosterone to 18-hydroxycorticosterone in serum. Defects in CYP11B2 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. Protein type: Mitochondrial; EC 1.14.15.5; Oxidoreductase; EC 1.14.15.4; Lipid Metabolism - androgen and estrogen; Lipid Metabolism - C21-steroid hormone. Chromosomal Location of Human Ortholog: 8q21-q22. Cellular Component: mitochondrion; mitochondrial inner membrane. Molecular Function: corticosterone 18-monooxygenase activity; steroid 11-beta-monooxygenase activity; iron ion binding; heme binding. Biological Process: steroid metabolic process; xenobiotic metabolic process; mineralocorticoid biosynthetic process; C21-steroid hormone biosynthetic process; renal water homeostasis; aldosterone mediated regulation of blood volume; sodium ion homeostasis; sterol metabolic process; aldosterone biosynthetic process; potassium ion homeostasis; cellular response to hormone stimulus. Disease: Corticosterone Methyloxidase Type Ii Deficiency; Corticosterone Methyloxidase Type I Deficiency