Beta-Galactosidase was partially restored by protease inhibitors, leupeptin, chymostatin and E-64 in cultured fibroblasts from three patients with beta-galactosidase-neuraminidase deficiency. Pepstatin did not activate this enzyme. Neuraminidase was not affected by any of these compounds in the culture medium. It was concluded that the activating effect was produced by a specific inhibition of thiol proteases.
Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors. Publishing Authors By Initials
Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors. Journal Published:
PUBLICATION TYPE: Research Support, Non-U.S. Gov
Journal: Journal of biochemistry
VOLUME: 90
Page Numbers: 271-3
Journal Abbreviation: J. Biochem.
ISSN: 0021-924X
DAY: 19
MONTH: Jul
YEAR: 1981
Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors. Information
Number of References:
LANGUAGE: eng
NlmUniqueID: 376600
Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors. Keywords Mesh Terms:
KEYWORDS: beta-Galactosidase
MESH TERMS: metabolism
Chemical & Substance for Abstract: Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors. Information
Substance Name: beta-Galactosidase
Registry Number: EC 3.2.1.23
Grant and Affiliation Information for Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors.
AFFILIATION:
Country: JAPAN
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MEDLINETA: J Biochem
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