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CYP11B2 Rabbit pAb (bs-10161R)  
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產品編號 bs-10161R
英文名稱 CYP11B2 Rabbit pAb
中文名稱 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.  
研究領域 心血管  細胞生物  免疫學  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse,Rat (predicted: Rabbit)
產品應用 WB=1:100-500,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 58 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYP11B2: 251-350/503 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P19099

Gene ID:
1585

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



產品圖片
Sample: Lane 1: Adrenal gland (Mouse) Tissue Lysate at 40 ug Lane 2: Adrenal gland (Rat) TissueLysate at 40 ug Primary: Anti-CYP11B2 (bs-10161R) at 1/1000 dilution Anti-beta-Actin (bs-0061R) at 1/2000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 58 kD Observed band size: 60 kD
Tissue/cell: human stomach tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-CYP11B2 Polyclonal Antibody, Unconjugated(bs-10161R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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