Patent ID: 8476414

Claim:
A substrate for mass spectrometric detection of a lysosomal storage disorder having the formula: A—(B1—B2—B3) (I) where A is D-glucose or D-galactose, B1 is C 1 alkyl or a C6 aryl having a N or O substituent extending from the ring, B2 is an amidyl terminating with a quaternary ammonium or carnitinyl, and B3 is an alkenyl alcohol with a carbon length of 12 to 20 or C 2 -C 20 alkyl having a substituent of O present as a carbonyl, where B1 is covalently associated with A by a glycosidic bond; said B1 is covalently associated with said A and said B2, and said B3 is covalently associated with said B2.