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Rabbit Anti-Steroid sulfatase/BF594 Conjugated antibody (bs-3857R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-3857R-BF594
英文名稱1 Rabbit Anti-Steroid sulfatase/BF594 Conjugated antibody
中文名稱 BF594標記的類固醇硫酸酯酶抗體
別    名 ARSC; ARSC1; Arylsulfatase C; Arylsulfatase C isozyme S; ASC; EC 3.1.6.2; ES; Estrone sulfatase; SSDD; Steroid sulfatase (microsomal); Steroid sulfatase (microsomal) arylsulfatase C isozyme S; Steroid sulfatase; Steryl sulfatase; Steryl sulfatase precursor; Steryl sulfate sulfohydrolase; STS; STS_HUMAN; Steryl-sulfatase; ASC; Steryl-sulfate sulfohydrolase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  免疫學(xué)  染色質(zhì)和核信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 62kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Steroid sulfatase C-terminus
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.
產(chǎn)品介紹 background:
The protein encoded by this gene catalyzes the conversion of sulfated steroid precursors to estrogens during pregnancy. The encoded protein is found in the endoplasmic reticulum, where it acts as a homodimer. Mutations in this gene are known to cause X-linked ichthyosis (XLI). [provided by RefSeq, Jul 2008].

Function:
Conversion of sulfated steroid precursors to estrogens during pregnancy.

Subunit:
Homodimer.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Post-translational modifications:
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.

DISEASE:
Defects in STS are the cause of ichthyosis X-linked (IXL) [MIM:308100]. Ichthyosis X-linked is a keratinization disorder manifesting with mild erythroderma and generalized exfoliation of the skin within a few weeks after birth. Affected boys later develop large, polygonal, dark brown scales, especially on the neck, extremities, trunk, and buttocks.

Similarity:
Belongs to the sulfatase family.

Database links:

Entrez Gene: 412 Human

Omim: 308100 Human

SwissProt: P08842 Human

Unigene: 522578 Human

Unigene: 700558 Human

Unigene: 700559 Human



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

類固醇硫酸酯酶SSDD缺乏,可導(dǎo)致病人的皮膚培養(yǎng)的纖維母細咆、滋養(yǎng)層細胞、外周白細胞、毛球的角化組織、表皮細胞、角質(zhì)層和甲等變化,見于X連鎖魚鱗病(XLI)。
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