| 货号 | 16089-100mg |
| 描述 | Cholesterol ester storage disease and Wolman disease are recessive autosomal disorders caused by a deficiency in lysosomal acid lipase (LAL), also known as cholesteryl ester hydrolase.1,2 4-Methylumbelliferyl palmitate (4-MUP) is a fluorogenic substrate for LAL.3 It may also be cleaved by other acid lipases.4,5 Recent advances allow the assessment of LAL activity in very small blood volumes using 4-MUP.1,2 |
| 别名 | 4-MUP;4-MU Palmitate;Palmitoyl 4-Methylumbelliferone; |
| 供应商 | Cayman |
| 应用文献 | |
| 1.Dairaku, T.,Iwamoto, T.,Nishimura, M., et al. A practical fluorometric assay method to measure lysosomal acid lipase activity in dried blood spots for the screening of cholesteryl ester storage disease and Wolman disease. Molecular Genetics and Metabolism 111(2), 193-196 (2014). 2.Hamilton, J.,Jones, I.,Srivastava, R., et al. A new method for the measurement of lysosomal acid lipase in dried blood spots using the inhibitor Lalistat 2. Clinica Chimica Acta 413(15-16), 1207-1210 (2012). 3.Kelly, S. and Bakhru-Kishore, R. Fluorimetric assay of acid lipase in human leukocytes. Clinica Chimica Acta 97(2-3), 239-242 (1979). 4.Warner, T.G.,Dambach, L.M.,Shim, J.H., et al. Separation and characterization of the acid lipase and neutral esterases from human liver. American Journal of Human Genetics 32(6), 869-879 (1980). 5.Baillargeon, M.W. and McCarthy, S.G. Geotrichum candidum NRRL Y-553 lipase: Purification, characterization and fatty acid specificity. Lipids 26(10), 831-836 (1991). | |
| 运输条件 | Room temperature in continental US; may vary elsewhere |
| 存放说明 | -20 |
| 纯度 | ≥98% |
| 计算分子量 | 414.6 |
| 分子式 | C26H38O4 |
| CAS号 | 17695-48-6 |
| 稳定性 | ≥ 2 years |
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