The cytoskeleton in Chediak-Higashi syndrome fibroblasts

R. E. Ostlund, R. W. Tucker, J. T. Leung, N. Okun, J. R. Williamson

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

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 perinuclear 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.

Original languageEnglish
Pages (from-to)806-811
Number of pages6
JournalUnknown Journal
Volume56
Issue number5
DOIs
StatePublished - 1980

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