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  • Title: Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies.
    Author: Tsuji D, Higashine Y, Matsuoka K, Sakuraba H, Itoh K.
    Journal: Clin Chim Acta; 2007 Mar; 378(1-2):38-41. PubMed ID: 17196574.
    Abstract:
    BACKGROUND: GM2 gangliosidoses, including Tay-Sachs disease, Sandhoff disease and the AB variant, comprise deficiencies of beta-hexosaminidase isozymes and GM2 ganglioside activator protein associated with accumulation of GM2 ganglioside (GM2) in lysosomes and neurosomatic clinical manifestations. A simple assay system for intracellular quantification of GM2 is required to evaluate the therapeutic effects on GM2-gangliosidoses. METHODS: We newly established a cell-ELISA system involving anti-GM2 monoclonal antibodies for measuring GM2 storage in fibroblasts from Tay-Sachs and Sandhoff disease patients. RESULTS: We succeeded in detecting the corrective effect of enzyme replacement on elimination of GM2 in the cells with this ELISA system. CONCLUSIONS: This simple and sensitive system should be useful as additional diagnosis tool as well as therapeutic evaluation of GM2 gangliosidoses.
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