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Rabbit Anti-HSD3B2/BF594 Conjugated antibody (bs-24205R-BF594)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-24205R-BF594
英文名稱(chēng) Rabbit Anti-HSD3B2/BF594 Conjugated antibody
中文名稱(chēng) BF594標(biāo)記的2型腎上腺皮質(zhì)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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  生長(zhǎng)因子和激素  新陳代謝  線(xiàn)粒體  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Mouse, 
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 40kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from mouse HSD3B2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
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:
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.

Database links:

Entrez Gene: 3283 Huma

Entrez Gene: 3284 Human

Entrez Gene: 15492 Mouse

Entrez Gene: 15493 Mouse

Entrez Gene: 15494 Mouse

Entrez Gene: 29632 Rat

Entrez Gene: 360348 Rat

Entrez Gene: 682974 Rat

Omim: 201810 Human

SwissProt: P14060 Human

SwissProt: P26439 Human

SwissProt: O35469 Mouse

SwissProt: P24815 Mouse

SwissProt: P26149 Mouse

SwissProt: P26150 Mouse

SwissProt: P22071 Rat

SwissProt: P22072 Rat

SwissProt: Q62878 Rat

Unigene: 364941 Human

Unigene: 654399 Human

Unigene: 140811 Mouse

Unigene: 14435 Mouse

Unigene: 158717 Mouse

Unigene: 482364 Mouse

Unigene: 109394 Rat

Unigene: 128814 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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