4-Methylumbelliferyl 6-thio-Palmitate-β-D-Glucopyranoside
货号：
19524-500ug 基本售价：
518.0 元 规格：
500 ug
产品信息
概述| 货号 | 19524-500ug |
| 描述 | 4-Methylumbelliferyl 6-thio-palmitate-β-D-glucopyranoside is a fluorogenic substrate of palmitoyl-protein thioesterase (PPT, also known as CLN1), a lysosomal hydrolase that removes long-chain fatty acyl groups from modified cysteine residues in proteins. This substrate is used in assays that measure PPT activity, which is commonly deficient in the neurodegenerative disorder known as infantile neuronal ceroid lipofuscinosis.1,2 |
| 别名 | Mu-6S-Palm-β-Glc; |
性能| 供应商 | Cayman |
| 应用文献 |
1.Das, A.K.,Lu, J.Y., and Hofmann, S.L. Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis. Human Molecular Genetics 10(13), 1431-1439 (2001).
2.Vozni, Y.V.,Keulemans, J.L.M.,Mancini, G.M.S., et al. A new simple enzyme assay for pre- and postnatal diagnosis of infantile neuronal ceroid lipofuscinosis (INCL) and its variants. Journal of Medical Genetics 36(6), 471-474 (1999).
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| 运输条件 | Room temperature in continental US; may vary elsewhere |
| 存放说明 | -20 |
| 纯度 | ≥95% |
| 计算分子量 | 592.8 |
| 分子式 | C32H48O8S |
| CAS号 | 229644-17-1 |
| 稳定性 | ≥ 2 years |
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