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Mant-m3gn2-dnp is a cytosolic enzyme that catalyses the deglycosylation of N-linked oligoglucans. It is used for the diagnosis of diseases involving glycosylase deficiency, such as Tay-Sachs disease and Sandhoff disease. This enzyme has also been used to diagnose disorders in which the synthesis of glycoproteins is altered, such as Gaucher disease and Niemann-Pick disease type C. Biological samples, including cerebrospinal fluid (CSF), are used to assay for Mant-m3gn2-dnp activity. The CSF can be obtained from a lumbar puncture or by intracerebroventricular injection of mice with human immunoglobulin G (IgG). A virus or vector containing the gene encoding Mant-m3gn2-dnp can be injected into a catheter placed in a ventricle of the brain. The vector provides an
