英文名稱
4-Methylumbelliferyl 6-Sulfo-2-acetamido-2-deoxy-(beta)-D-glucopyranoside Potassium Salt
純度
>95% (HPLC)
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Methanol (Slightly), Water (Slightly)
Standards; Fluorescent Reagents; Carbohydrates and Oligosaccharides;
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.