Patent ID: 8431335

Claim:
An assay mix for determining the activity of lysosomal acid α-glucosidase comprising (7-benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]-ethyl}-carbamic acid tert-butyl ester, (7-d5-benzoylamino-heptyl)-[2-(4-hydroxy-phenylcarbamoyl)-ethyl]-carbamic acid tert-butyl ester, 3-[(3-cholamidopropyl)dimethylammonio]-1-propanesulfonate (“CHAPS”), acarbose and a buffer adjusted to a pH of 4.0, wherein the ratio of (7-benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]ethyl}-carbamic acid tert-butyl ester to (7-d5-benzoylamino-heptyl)-[2-(4-hydroxy-phenylcarbamoyl)-ethyl]-carbamic acid tert-butyl ester is 100:1, the ratio of CHAPS to (7-benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]-ethyl}-carbamic acid tert-butyl ester is 24.3:1, the ratio of acarbose to (7-benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]-ethyl}-carbamic acid tert-butyl ester is 0.02:1, and the ratio of buffer to (7-benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]ethyl}-carbamic acid tert-butyl ester is 450:1.