4-Methylumbelliferyl 6-Sulfo-2-acetamido-2-deoxy-b-D-glucopyranoside, Potassium Salt

Chemical Properties

White Crystalline Solid

Uses

4-Methylumbelliferyl 6-Sulfo-2-acetamido-2-deoxy-b-D-glucopyranoside, potassium salt is a fluorogenic substrate used for 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. They include Tay Sachs disease, Sandhoff disease and other forms.

Uses

A fluorogenic substrate used for 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. They include Tay Sachs disease, Sandhoff disease and other forms.