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

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.

• Authors: A L Miller, R Stein, M Sundsmo, R Y Yeh
• Journal: The Biochemical journal (Biochem J) Vol. 238 Issue 2 Pg. 589-95 (Sep 01 1986) ISSN: 0264-6021 [Print] England
• PMID: 3099770 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Hydrolases
• Mannosidases
• beta-Glucosidase
• alpha-Mannosidase

Topics

• Cells, Cultured
• Chediak-Higashi Syndrome (enzymology)
• Child, Preschool
• Endocytosis
• Female
• Fibroblasts (enzymology)
• Humans
• Hydrolases (metabolism)
• Infant
• Kinetics
• Lysosomes (enzymology)
• Male
• Mannosidases (metabolism)
• alpha-Mannosidase
• beta-Glucosidase (metabolism)