Hexosaminidase C in Brain Affected by Tay-Sachs Disease

Abstract

MINAMI, R., NAKAMURA, F., OYANAGI, K, and NAKAO, T. Hexosaminidase C in Brain Affected by Tay-Sachs Disease. Tohoku J. exp. Med., 1981, 133 (2), 175-185-Hexosaminidase (Hex) C could be detected in human brains by DEAE cellulose column chromatography, when N-acetyl-β-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.