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Rabbit Anti-CYP11B1/AP Conjugated antibody (bs-23821R-AP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-23821R-AP
英文名稱 Rabbit Anti-CYP11B1/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的細胞色素P450 11B1抗體
別    名 CPN1; CYP11B; CYPXIB1; Cytochrome P450 11B1, mitochondrial; Cytochrome p450 XIB1; Cytochrome P450, family 11, subfamily B, polypeptide 1; FHI; P 450C11; P450C11; S11BH; Steroid 11 beta hydroxylase; Steroid 11 beta monooxygenase; Q3TG86_MOUSE; Cytochrome P-450c11; Cytochrome P450C11; Steroid 11-beta-hydroxylase.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from mouse CYP11B1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 Constituents: 0.05M TBS, pH 8.0 with 10mg/ml BSA and 0.05% NaN3, 50% glycerol. Or Lyophilized. Buffer = 0.05M TBS, pH 8.0 with 10mg/ml BSA and 0.05% NaN3. Reconstitute with sterile distilled water.
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 and 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.

Function:
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.

Subcellular Location:
Mitochondrion membrane.

DISEASE:
Defects in CYP11B1 are the cause of adrenal hyperplasia type 4 (AH4) [MIM:202010]. 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 adult 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 hyperaldosteronism familial 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: 1584 Human

Entrez Gene: 110115 Mouse

Omim: 610613 Human

SwissProt: P15538 Human

SwissProt: Q3TG86 Mouse

Unigene: 184927 Human




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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