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  Vol. 39 No. 9, September 1982 TABLE OF CONTENTS
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Brain glucocerebrosidase in Gaucher's disease

L. B. Daniels, P. J. Coyle, R. H. Glew, N. S. Radin and R. S. Labow

Using glucocerebroside labeled with carbon 14 as the substrate, we determined that homogenates of brain tissue from both neuropathic and nonneuropathic cases of Gaucher's disease were profoundly deficient (more than 85%) in glucocerebrosidase activity. The beta-glucosidase activity, as measured with 4-methylumbelliferyl-beta-D-glucopyranoside as the substrate, in the homogenates of brain from four cases of Gaucher's disease was less sensitive to inhibition by conduritol B epoxide (CBE) when compared with normal brain beta-glucosidase. However, when homogenates were assayed with radiolabeled glucocerebroside as the substrate, no differential sensitivity toward CBE was indicated, suggesting the presence of an additional, CBE-insensitive, beta-glucosidase in brain tissue. Residual glucocerebrosidase activity partially purified from the brain of an adult with type 1 Gaucher's disease was activated threefold by gluconoyl hydrazine, whereas the same enzyme from control brain was unaffected, and eight times less sensitive to gluconolactone inhibition.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Viable Mouse Models of Acid {beta}-Glucosidase Deficiency: The Defect in Gaucher Disease
Xu et al.
Am. J. Pathol. 2003;163:2093-2101.
ABSTRACT | FULL TEXT  





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