Characterization of lysosomes and lysosomal enzymes from Chediak-Higashi-syndrome cultured fibroblasts
- 1 September 1986
- journal article
- research article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 238 (2) , 589-595
- https://doi.org/10.1042/bj2380589
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.This publication has 33 references indexed in Scilit:
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