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上海泛柯实业有限公司
FITC标记的芳香基硫酸酯酶1抗体产品介绍:This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
英文名称 | Anti-ARSA/FITC |
中文名称 | FITC标记的芳香基硫酸酯酶1抗体 |
别 名 | As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arsA; ARSA_HUMAN; arylsulfatase A; Arylsulfatase A component C; AS A; Cerebroside-sulfatase; MGC125207; MLD. |
说 明 书 | 100ul |
研究领域 | 肿瘤 细胞生物 神经生物学 细胞自噬 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Human, Mouse, Rat, |
产品应用 | 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 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human ARSA |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
储 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 |
保存条件 | 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. |
产品介绍 | 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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