4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) (4-MU-Gal-6S) is a fluorogenic substrate used to quantify N-acetylgalactosamine-6-sulphatase (GALNS) activity.
CAS：206443-06-3
分子式：C16H17O11S.Na
分子量：440.4
纯度：98%
存储：Store at -20°C

Background:

4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) (4-MU-Gal-6S) is a fluorogenic substrate used to quantify N-acetylgalactosamine-6-sulphatase (GALNS) activity^[1]. 4-MU-Gal-6S is cleaved by GALNS to release the fluorescent moiety 4-MU. 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high (7.12-10.3) pH, respectively, and an emission maximum ranging from 445 to 455 nM, increasing as pH decreases^[2]. It has been used to detect Morquio disease type A, a lysosomal storage disorder in which GALNS is deficient^[1]. 4-MU-Gal-6S can be used to assess GALNS activity in a very small blood volume to determine the extent of deficiency.^[3]

参考文献：

1. Van Diggelen, O.P., Zhao, H., Kleijer, W.J., et al. A fluorimetric enzyme assay for the diagnosis of Morquio disease type A (MPS IV A). Clin. Chim. Acta 187(2), 131-139 (1990).

2. Zhi, H., Wang, J., Wang, S., et al. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule. J. Spectrosc. 1(1), 147128 (2013).

3. Cozma, C., Eichler, S., Wittmann, G., et al. Diagnosis of Morquio syndrome in dried blood spots based on a new MRM-MS assay. PLoS One 10(7), (2015).