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The cytoskeleton in Chediak-Higashi syndrome fibroblasts

Authors :
Ostlund, RE Jr
Tucker, RW
Leung, JT
Okun, N
Williamson, JR
Source :
Blood; November 1980, Vol. 56 Issue: 5 p806-811, 6p
Publication Year :
1980

Abstract

The Chediak-Higashi syndrome (CHS) trait is expressed in cultured human skin fibroblasts as an abnormal perinuclear concentration of moderately enlarged lysosomes. The cytoskeleton of CHS fibroblasts appears intact. Microtubules are normal in number and morphology, as assessed by colchicine binding studies, antitubulin immunofluorescence, and electron microscopy. Deformability by shear force is unaltered and microfilaments are abundant. However, CHS lysosomes appear to interact abnormally with the cytoskeleton, since the perinculear aggregation partially disperses after depolymerization of cell microtubules with colchicine. These results suggest that CHS is associated with a defect of either the lysosomal membrane itself or of lysosomal membrane- microtubule interaction.

Details

Language :
English
ISSN :
00064971 and 15280020
Volume :
56
Issue :
5
Database :
Supplemental Index
Journal :
Blood
Publication Type :
Periodical
Accession number :
ejs52891440
Full Text :
https://doi.org/10.1182/blood.V56.5.806.806