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Rabbit Anti-StAR/BF594 Conjugated antibody (bs-20388R-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-20388R-BF594
英文名稱(chēng)1 Rabbit Anti-StAR/BF594 Conjugated antibody
中文名稱(chēng) BF594標(biāo)記的促黃體激素誘導(dǎo)蛋白抗體
別    名 StARD1; Cholesterol trafficker; Luteinizing hormone induced protein; Mitochondrial steroid acute regulatory protein; StAR related lipid transfer (START) domain containing 1; StARD1; START domain containing protein 1; Steroidogenic Acute Regulatory Protein; Steroidogenic acute regulatory protein mitochondrial; STAR_HUMAN.  
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 腫瘤  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  激酶和磷酸酶  線粒體  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
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 32kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human StAR
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:
The protein encoded by this gene plays a key role in the acute regulation of steroid hormone synthesis by enhancing the conversion of cholesterol into pregnenolone. This protein permits the cleavage of cholesterol into pregnenolone by mediating the transport of cholesterol from the outer mitochondrial membrane to the inner mitochondrial membrane. Mutations in this gene are a cause of congenital lipoid adrenal hyperplasia (CLAH), also called lipoid CAH. A pseudogene of this gene is located on chromosome 13. [provided by RefSeq, Jul 2008].

Function:
Plays a key role in steroid hormone synthesis by enhancing the metabolism of cholesterol into pregnenolone. Mediates the transfer of cholesterol from the outer mitochondrial membrane to the inner mitochondrial membrane where it is cleaved to pregnenolone.

Subunit:
May interact with TSPO.

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in STAR are the cause of adrenal hyperplasia type 1 (AH1) [MIM:201710]. The most severe form of adrenal hyperplasia. It is a condition characterized by onset of profound adrenocortical insufficiency shortly after birth, hyperpigmentation reflecting increased production of pro-opiomelanocortin, elevated plasma rennin activity as a consequence of reduced aldosterone synthesis, and male pseudohermaphroditism resulting from deficient fetal testicular testosterone synthesis. Affected individuals are phenotypic females irrespective of gonadal sex, and frequently die in infancy if mineralocorticoid and glucocorticoid replacement are not instituted.

Similarity:
Contains 1 START domain.

Database links:

Entrez Gene: 6770 Human

Entrez Gene: 20845 Mouse

Entrez Gene: 25557 Rat

Omim: 600617 Human

SwissProt: P49675 Human

SwissProt: P51557 Mouse

SwissProt: P97826 Rat

Unigene: 521535 Human

Unigene: 293314 Mouse

Unigene: 11399 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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