206443-06-3 , 4-Methylumbelliferyl b-D-galactopyranoside-6-sulphate sodium salt

206443-06-3 , 4-Methylumbelliferyl b-D-galactopyranoside-6-sulphate sodium salt
C16H17NaO11S / 440.36
MFCD01861961

4-Methylumbelliferyl b-D-galactopyranoside-6-sulphate sodium salt

A substrate used for the diagnosis of Morquio disease type A.

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.

CAS Number	206443-06-3
Product Name	Sodium;[(2R,3R,4S,5R,6S)-3,4,5-trihydroxy-6-(4-methyl-2-oxochromen-7-yl)oxyoxan-2-yl]methyl sulfate
IUPAC Name	sodium;[(2R,3R,4S,5R,6S)-3,4,5-trihydroxy-6-(4-methyl-2-oxochromen-7-yl)oxyoxan-2-yl]methyl sulfate
Molecular Formula	C16H17NaO11S
Molecular Weight	440.4
InChI	InChI=1S/C16H18O11S.Na/c1-7-4-12(17)26-10-5-8(2-3-9(7)10)25-16-15(20)14(19)13(18)11(27-16)6-24-28(21,22)23;/h2-5,11,13-16,18-20H,6H2,1H3,(H,21,22,23);/q;+1/p-1/t11-,13+,14+,15-,16-;/m1./s1
InChI Key	KHTOBGQRIASILC-XNSOHGFJSA-M
SMILES	CC1=CC(=O)OC2=C1C=CC(=C2)OC3C(C(C(C(O3)COS(=O)(=O)[O-])O)O)O.[Na+]
Synonyms	4-Methyl-7-[(6-O-sulfo-β-D-galactopyranosyl)oxy]-2H-1-benzopyran-2-one Monosodium Salt;