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  • Title: Hexosaminidase C in brain affected by Tay-Sachs Disease.
    Author: Minami R, Nakamura F, Oyanagi K, Nakao T.
    Journal: Tohoku J Exp Med; 1981 Feb; 133(2):175-85. PubMed ID: 7268761.
    Abstract:
    Hexosaminidase (Hex) C could be detected in human brains by DEAE cellulose column chromatography, when N-acetyl-beta-D-glucosaminidase activity was determined at pH 5.5. In the chromatographic pattern, the peak of Hex C was always higher than that of Hex A in control prenatal brains and conversely lower in postnatal brains. Hex C was not detected in the brains obtained from two cases of Tay-Sachs disease. In addition, the content of Hex C in the brains of two other Tay-Sachs cases was lower than that of the control. The observation suggests that the absence or reduction of Hex C may have some relation with biochemical defects in Tay-Sachs disease, although Hex C does not share common subunit structures with Hex A and the role of Hex C with respect to GM2-ganglioside degradation remains unknown yet.
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