產(chǎn)品編號(hào) | bsm-51534M |
英文名稱 | Mouse Anti-ARSA antibody |
中文名稱 | ARSA單克隆抗體 |
別 名 | As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arylsulfatase A; AS A; MLD. |
研究領(lǐng)域 | 免疫學(xué) 神經(jīng)生物學(xué) |
抗體來(lái)源 | Mouse |
克隆類型 | Monoclonal |
克 隆 號(hào) | S9F5 |
交叉反應(yīng) | Human |
產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
細(xì)胞定位 | 細(xì)胞漿 |
性 狀 | Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human ARSA: 401-507/507 |
亞 型 | IgG2a |
純化方法 | affinity purified by Protein G |
緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
產(chǎn)品介紹 |
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 ultimately 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). Similarity: Belongs to the sulfatase family. Contains 2 SH2 domains. SWISS: P15289 Gene ID: 410 Database links: Entrez Gene: 410 Human SwissProt: P15289 Human |
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