Methods are provided for detecting enzymatic activity of various lysosomal storage enzymes using substrates that include: a sugar moiety; a linker moiety allowing the conjugation of sugar moiety with the remaining structure of the substrate; and two or more fatty acid chains or derivatives thereof at least one of which is sufficiently structured to provide improved solubility in aqueous or organic solvent systems. Also provided are internal standards, and inhibitors for use in detecting or reducing enzymatic activity using the inventive substrates.
提供了一种检测各种溶酶体贮存酶的酶活性的方法,使用的底物包括:糖基;连接基团,允许糖基与底物的其余结构结合;以及至少两个
脂肪酸链或其衍
生物,其中至少一个足够结构化以提供在
水性或有机溶剂体系中改善溶解度。还提供了内部标准和
抑制剂,用于使用创新底物检测或减少酶活性。