CYP11B2 Mouse Monoclonal Antibody(ARM652)
Key features and details
- Target:
- Clone ID:
- Host:
- Species Cross-reactivity:
- Applications:
- Dilution:
- Clonality:
-
Brand:
CAT.NO. : ARM6943
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Product Details
Background
Defects in CYP11B2 are the cause of corticosterone methyloxidase type 1 deficiency (CMO-1 deficiency) [MIM:203400]; 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) [MIM:610600]. 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) [MIM:103900]. 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. Note=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.
Application
|
Application |
Dilution Ratio |
|
IHC |
1:50 - 1:200 |
Overview
|
Host |
Mouse |
|
Species Reactivity |
Reacts with: Human |
|
Immunogen |
Synthetic peptide corresponding to Human C11B2/CYP11B2 aa 1-100 |
|
Positive Control |
Human adrenal gland lysate; Human kidney and liver tissues |
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Form |
Liquid |
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Purity |
Protein A purified |
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Clonality |
Monoclonal |
|
Isotype |
IgG |
Data
Storage
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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