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聯(lián)系我時(shí),請告知來自 智慧城市網(wǎng)FITC標(biāo)記的芳香基硫酸酯酶1抗體產(chǎn)品介紹:This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
英文名稱 | Anti-ARSA/FITC |
中文名稱 | FITC標(biāo)記的芳香基硫酸酯酶1抗體 |
別 名 | As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arsA; ARSA_HUMAN; arylsulfatase A; Arylsulfatase A component C; AS A; Cerebroside-sulfatase; MGC125207; MLD. |
說 明 書 | 100ul |
研究領(lǐng)域 | 腫瘤 細(xì)胞生物 神經(jīng)生物學(xué) 細(xì)胞自噬 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | Human, Mouse, Rat, |
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 | 47/54kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human ARSA |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 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: The protein encoded by this gene hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimay death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010]. Function: Hydrolyzes cerebroside sulfate. Subunit: Homodimer at neutral pH and homooctamer at acidic pH. Exists both as a single chain of 58 kDa (component A) or as a chain of 50 kDa (component B) linked by disulfide bond(s) to a 7 kDa chain (component C). Interacts with SUMF1. Subcellular Location: Lysosome. 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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD). |
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