206443-06-3

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. 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. It has been used to detect Morquio disease type A, a lysosomal storage disorder in which GALNS is deficient. 4-MU-Gal-6S can be used to assess GALNS activity in a very small blood volume to determine the extent of deficiency.

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4-Methylumbelliferylb-D-galactopyranoside-6-sulphatesodiumsalt is used for the determination of galactose-6-sulphate sulphatase activity,it is used in the diagnosis of Mucopolysaccharidosis IV A.

Used for the determination of galactose-6-sulphate sulphatase activity. Used in the diagnosis of Mucopolysaccharidosis IV A.

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[1] O.P. VAN DIGGELEN . A fluorimetric enzyme assay for the diagnosis of Morquio disease type A (MPS IV A)[J]. Clinica Chimica Acta, 1990, 187 2: Pages 131-139. DOI: 10.1016/0009-8981(90)90339-t
[2] CLAUDIA COZMA. Diagnosis of Morquio Syndrome in Dried Blood Spots Based on a New MRM-MS Assay.[J]. PLoS ONE, 2015: e0131228. DOI: 10.1371/journal.pone.0131228