| 產(chǎn)品編號(hào) |
bs-10161R-BF647 |
| 英文名稱1 |
Rabbit Anti-CYP11B2/BF647 Conjugated antibody
|
| 中文名稱 |
BF647標(biāo)記的醛固酮合成酶CYP11B2抗體 |
| 別 名 |
CYP 11B2; CYPXI11B2; Cytochrome P450 1111B2; Cytochrome P450 1111B2 mitochondrial; Cytochrome P450 family 11 subfamily B polypeptide 2; Cytochrome P450 subfamily XIB (cholesterol side chain cleavage); Cytochrome P450 subfamily XI11B2; Cytochrome P450C1111B2; C11B2_HUMAN. |
| 規(guī)格價(jià)格 |
100ul/2980元
購(gòu)買(mǎi) 大包裝/詢價(jià) |
| 說(shuō) 明 書(shū) |
100ul
|
| 研究領(lǐng)域 |
心血管 細(xì)胞生物 免疫學(xué) 線粒體 |
| 抗體來(lái)源 |
Rabbit |
| 克隆類型 |
Polyclonal |
| 交叉反應(yīng) |
Human,
(predicted: Mouse, Rat, Rabbit, )
|
| 產(chǎn)品應(yīng)用 |
ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 |
58kDa |
| 性 狀 |
Lyophilized or Liquid |
| 濃 度 |
1mg/ml |
| 免 疫 原 |
KLH conjugated synthetic peptide derived from human CYP11B2 |
| 亞 型 |
IgG |
| 純化方法 |
affinity purified by Protein A |
| 儲(chǔ) 存 液 |
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 |
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background:
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].
Function:
Preferentially catalyzes the conversion of 11-deoxycorticosterone to aldosterone via corticosterone and 18-hydroxycorticosterone.
Subcellular Location:
Mitochondrion membrane.
DISEASE:
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.
Similarity:
Belongs to the cytochrome P450 family.
Database links:
Entrez Gene: 1585 Human
Entrez Gene: 13072 Mouse
Entrez Gene: 24294 Rat
Omim: 124080 Human
SwissProt: P19099 Human
SwissProt: P15539 Mouse
SwissProt: P30099 Rat
Unigene: 632054 Human
Unigene: 377079 Mouse
Unigene: 144549 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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