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| カタログ番号 | HY292016 |
|---|---|
| 種反応性 | Human |
| アプリケーション | ELISA, Functional assay, Research in vivo |
| アイソタイプ | Human lysosomal acid glucosylceramidase |
| 発現システム | Mammalian cells |
| ターゲット | SGTase, Cholesterol glucosyltransferase, GBA, Cholesteryl-beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, GLUC, Imiglucerase, Beta-glucocerebrosidase, Lysosomal acid GCase, Beta-GC, Alglucerase, GC, Lysosomal acid glucosylceramidase, Acid beta-glucosidase |
| エンドトキシンレベル | < 10 EU/mg |
| 純度 | >95% purity as determined by SDS-PAGE. |
| 精製 | Protein A/G purified from cell culture supernatant. |
| アクセッション番号 | P04062 |
| 形態 | Liquid |
| 保存バッファー | 0.01M PBS pH 7.4 データシートのハードコピーまたはロット固有のCOAに記載された具体的なバッファー情報を参照してください。 |
| 安定性と保存 | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. |
| 別名 | 2725857-55-4 |
| 背景 | Lysosomal acid glucosylceramidase (GBA) is a ~59 kDa protein. Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose. Plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation. Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol. GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine). 1. Ron, I. et al. (2005) Blood cells, molecules & diseases 35, 57-65. PMID: 15916907 2. Akiyama, H. et al. (2013) Biochemical and biophysical research communications 441, 838-43. PMID: 24211208 3. Akiyama, H. et al. (2020) The Journal of biological chemistry 295, 5257-5277. PMID: 32144204 4. Bannink, S. et al. (2024) Journal of lipid research 65, 100670. PMID: 39395789 5. Vaccaro, AM. et al. (1997) The Journal of biological chemistry 272, 16862-7. PMID: 9201993 6. Magalhaes, J. et al. (2016) Human molecular genetics 25, 3432-3445. PMID: 27378698 7. Kitatani, K. et al. (2009) The Journal of biological chemistry 284, 12972-8. PMID: 19279011 8. Marques, AR. et al. (2016) Journal of lipid research 57, 451-63. PMID: 26724485 9. Boer, DE. et al. (2021) Journal of lipid research 62, 100018. PMID: 33361282 |
| 参考文献 | 1. Neal J Weinreb, et al. Imiglucerase and its use for the treatment of Gaucher's disease. Expert Opin Pharmacother. 2008 Aug;9(11):1987-2000. [HY292016] |
| 注意事項 | For research use only. Not suitable for clinical or therapeutic use. |
SDS-PAGE for Research Grade Velaglucerase Beta
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