Characterization of Lysosomes and Lysosomal Enzymes from Chediak-Higashi-syndrome Cultured Fibroblasts

Overview

Journal Biochem J

Specialty Biochemistry

Date 1986 Sep 1

PMID 3099770

Citations 2

Authors

A L Miller

R Stein

M Sundsmo

R Y Yeh

Affiliations

Soon will be listed here.

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.

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