產品貨號 :  mlR24205

英文名稱 :  HSD3B2

中文名稱 :  2型腎上腺皮質增生癥蛋白抗體

別    名 :  3 beta HSD adrenal and gonadal type; 3 beta HSD II; 3 beta HSD type II; 3 beta hydroxy 5 ene steroid dehydrogenase; 3 beta hydroxy Delta(5) steroid dehydrogenase; 3 beta hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 2; 3 beta-hydroxysteroid dehydrogenase type II, delta 5-delta 4-isomerase type II, 3 beta-HSD type II; 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type II; 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 2; 3-beta-HSD II; 3-beta-hydroxy-5-ene steroid dehydrogenase; 3-beta-hydroxy-Delta(5)-steroid dehydrogenase; 3BHS2_HUMAN; ADRENAL HYPERPLASIA II; beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 2; delta 5 delta 4 isomerase type II; Delta-5-3-ketosteroid isomerase; HSD3B; HSD3B2; HSDB; HSDB3B; hydroxy delta 5 steroid dehydrogenase, 3 beta and steroid delta isomerase 2; Hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2; Progesterone reductase.  

研究領域 :  細胞生物  信號轉導  生長因子和激素  新陳代謝  線粒體  

抗體來源 :  Rabbit

克隆類型 :  Polyclonal

交叉反應 :   Mouse,

產品應用 :  WB=1:500-2000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  40kDa

細胞定位 :  細胞漿 線粒體

性    狀 :  Lyophilized or Liquid

濃    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from mouse HSD3B2:151-250/372

亞    型 :  IgG

純化方法 :  affinity purified by Protein A

儲 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.

PubMed :  PubMed

產品介紹 : 3-beta-HSD is a bifunctional enzyme, that catalyzes the oxidative conversion of Delta(5)-ene-3-beta-hydroxy steroid, and the oxidative conversion of ketosteroids. The 3-beta-HSD enzymatic system plays a crucial role in the biosynthesis of all classes of hormonal steroids.

Function:

3-beta-HSD is a bifunctional enzyme, that catalyzes the oxidative conversion of Delta(5)-ene-3-beta-hydroxy steroid, and the oxidative conversion of ketosteroids. The 3-beta-HSD enzymatic system plays a crucial role in the biosynthesis of all classes of hormonal steroids.

Subunit:

Expressed in adrenal gland, testis and ovary.

Subcellular Location:

Endoplasmic reticulum membrane. Mitochondrion membrane.

Tissue Specificity:

Defects in HSD3B2 are the cause of adrenal hyperplasia type 2 (AH2) [MIM:201810]. AH2 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). In AH2, virilization is much less marked or does not occur. AH2 is frequently lethal in early life.

Note=Mild HSD3B2 deficiency in hyperandrogenic females is associated with characteristic traits of polycystic ovary syndrome, such as insulin resistance and luteinizing hormon hypersecretion.

DISEASE:

Defects in HSD3B2 are the cause of adrenal hyperplasia type 2 (AH2) [MIM:201810]. AH2 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). In AH2, virilization is much less marked or does not occur. AH2 is frequently lethal in early life.

Note=Mild HSD3B2 deficiency in hyperandrogenic females is associated with characteristic traits of polycystic ovary syndrome, such as insulin resistance and luteinizing hormon hypersecretion

Similarity:

Belongs to the 3-beta-HSD family.

SWISS:

P26149

Gene ID:

15493

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

產品圖片