Literature DB >> 6793566

Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors.

Y Suzuki, H Sakuraba, K Hayashi, K Suzuki, K Imahori.   

Abstract

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.

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Year:  1981        PMID： 6793566     DOI： 10.1093/oxfordjournals.jbchem.a133462

Source DB:  PubMed          Journal:  J Biochem        ISSN： 0021-924X            Impact factor:   3.387


  10 in total

Review 1.  The early and late processing of lysosomal enzymes: proteolysis and compartmentation.

Authors:  A Hasilik
Journal:  Experientia       Date:  1992-02-15

2.  Sialidosis and galactosialidosis: chromosomal assignment of two genes associated with neuraminidase-deficiency disorders.

Authors:  O T Mueller; W M Henry; L L Haley; M G Byers; R L Eddy; T B Shows
Journal:  Proc Natl Acad Sci U S A       Date:  1986-03       Impact factor: 11.205

3.  The effects of sucrose loading on lysosomal hydrolases.

Authors:  T Kato; S Okada; T Yutaka; H Yabuuchi
Journal:  Mol Cell Biochem       Date:  1984       Impact factor: 3.396

4.  GM1-Gangliosidosis: a molecular abnormality of acid beta-galactosidase in fibroblasts.

Authors:  T Furuya; Y Suzuki
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

5.  A severe infantile sialidosis (beta-galactosidase-alpha-neuraminidase deficiency) mimicking GM1-gangliosidosis type 1.

Authors:  S Okada; H Sugino; T Kato; T Yutaka; M Koike; T Dezawa; T Yamano; H Yabuuchi
Journal:  Eur J Pediatr       Date:  1983-09       Impact factor: 3.183

6.  Complementation of multiple sulfatase deficiency in somatic cell hybrids.

Authors:  K Fedde; A L Horwitz
Journal:  Am J Hum Genet       Date:  1984-05       Impact factor: 11.025

7.  Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.

Authors:  A D'Azzo; A Hoogeveen; A J Reuser; D Robinson; H Galjaard
Journal:  Proc Natl Acad Sci U S A       Date:  1982-08       Impact factor: 11.205

8.  Galactosialidosis: molecular heterogeneity in biosynthesis and processing of protective protein for beta-galactosidase.

Authors:  E Nanba; A Tsuji; K Omura; Y Suzuki
Journal:  Hum Genet       Date:  1988-12       Impact factor: 4.132

9.  Identification of targeting proteinase for rat alpha 1-macroglobulin in vivo. Mast-cell tryptase is a major component of the alpha 1-macroglobulin-proteinase complex endocytosed into rat liver lysosomes.

Authors:  A Tsuji; T Akamatsu; H Nagamune; Y Matsuda
Journal:  Biochem J       Date:  1994-02-15       Impact factor: 3.857

10.  Intracellular processing and maturation of mutant gene products in hereditary beta-galactosidase deficiency (beta-galactosidosis).

Authors:  A Oshima; K Yoshida; K Itoh; R Kase; H Sakuraba; Y Suzuki
Journal:  Hum Genet       Date:  1994-02       Impact factor: 4.132
  10 in total

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