4-Methylumbelliferylb-D-galactopyranoside-6-sulphatesodiumsalt C화학적 특성, 용도, 생산
개요
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.
화학적 성질
White Solid
용도
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.
4-Methylumbelliferylb-D-galactopyranoside-6-sulphatesodiumsalt 준비 용품 및 원자재
원자재
준비 용품
4-Methylumbelliferylb-D-galactopyranoside-6-sulphatesodiumsalt 공급 업체
