Description
MUGS is a fluorogenic substrate used in the diagnosis of GM2 gangliosidoses in leukocytes. Genetic defects in human b-hexosaminidase isozymes (Hex A & Hex B) result in various clinical and biochemical types of GM2 gangliosidosis (eg. Tay Sachs disease).
Applications
Fluorogenic substrate used for the diagnosis of GM2 gangliosidoses in leukocytes
Synonyms
7-[[2-(Acetylamino)-2-deoxy-6-O-sulfo-β-D-glucopyranosyl]oxy]-4-methyl-2H-1-benzopyran-2-one Monopotassium Salt
Molecular Formula
C18H20KNO11S
Melting Point
190-193°C (lit.)(dec.)