4-Methylumbelliferyl 2-deoxy-2-sulfamino-a-D-glucopyranoside sodium salt

4-Methylumbelliferyl N-sulpho-a-D-glucosaminide.Na; 4-MU-GlcNS; M3A; 4MU-a-N-sulpho-D-glucosaminide

A substrate used for the diagnosis of Sanfilippo disease type A (MPS IIIA).

4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (4-MU-α-GlcNS) is a fluorogenic substrate of heparin sulphamidase. Heparin sulphamidase cleaves 4-MU-α-GlcNS to yield 4-MU-α-GlcNH2, which is then cleaved by α-glucosaminidase to release the fluorescent product 4-MU, which displays an emission maxima of 445-454 nm. The excitation maxima for 4-MU is pH-dependent: 330, 370, and 385 nm at pH 4.6, 7.4, and 10.4, respectively. 4-MU-α-GlcNS has been used to quantify heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders.