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Literature DB >> 3099770

Characterization of lysosomes and lysosomal enzymes from Chediak-Higashi-syndrome cultured fibroblasts.

A L Miller, R Stein, M Sundsmo, R Y Yeh.

Abstract

Chediak-Higashi-syndrome cultured skin fibroblasts were used to study the possible involvement of lysosomal enzymes and lysosomal dysfunction in this disorder. Our evidence indicated that Chediak-Higashi fibroblasts displayed a significant decrease in the specific activity of the acidic alpha-D-mannosidase (pH 4.2) compared with normal controls. Additional studies revealed a small, but significant, decrease in the rate of degradation of 125I-labelled beta-D-glucosidase that had been endocytosed into Chediak-Higashi cells.

Entities: Disease

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Year: 1986 PMID： 3099770 PMCID： PMC1147173 DOI： 10.1042/bj2380589

Source DB: PubMed Journal: Biochem J ISSN： 0264-6021 Impact factor: 3.857

33 in total

1. Tissue fractionation studies. 15. Intracellular distribution and properties of beta-N-acetylglucosaminidase and beta-galactosidase in rat liver.

Authors: O Z SELLINGER; H BEAUFAY; P JACQUES; A DOYEN; C DE DUVE
Journal: Biochem J Date: 1960-03 Impact factor: 3.857

2. Congenital gigantism of peroxidase granules; the first case ever reported of qualitative abnormity of peroxidase.

Authors: O HIGASHI
Journal: Tohoku J Exp Med Date: 1954-02-25 Impact factor: 1.848

3. Radioiodination of proteins in single polyacrylamide gel slices. Tryptic peptide analysis of all the major members of complex multicomponent systems using microgram quantities of total protein.

Authors: J H Elder; R A Pickett; J Hampton; R A Lerner
Journal: J Biol Chem Date: 1977-09-25 Impact factor: 5.157

Characterization of lysosomes and lysosomal enzymes from Chediak-Higashi-syndrome cultured fibroblasts.

1. Tissue fractionation studies. 15. Intracellular distribution and properties of beta-N-acetylglucosaminidase and beta-galactosidase in rat liver.

2. Congenital gigantism of peroxidase granules; the first case ever reported of qualitative abnormity of peroxidase.

3. Radioiodination of proteins in single polyacrylamide gel slices. Tryptic peptide analysis of all the major members of complex multicomponent systems using microgram quantities of total protein.

4. The nature of the residual alpha-mannosidase in plasma in bovine mannosidosis.

5. The Chediak-Higashi syndrome: cytoplasmic sequestration in circulating leukocytes.

6. Lysosomal enzymes in normal and Chediak-Higashi blood leukocytes.

7. The Chediak-Higashi syndrome: a possible lysosomal disease.

8. The Chediak-Higashi syndrome: quantitation of a deficiency in maximal bactericidal capacity.

9. A hereditary alteration in kidneys of mice with Chediak-Higashi syndrome.

10. Lysosome enlargement in the Chediak-Higashi syndrome.

1. Properties of N-acetylglucosamine 1-phosphotransferase from human lymphoblasts.

2. The giant organelles in beige and Chediak-Higashi fibroblasts are derived from late endosomes and mature lysosomes.