Fibroblasts and macrophages of mice with the Chediak-Higashi-like syndrome have microtubules and actin cables
نویسندگان
چکیده
Cells of the beige mouse contain abnormally large lysosomes and show enhanced capping of concanavalin A. It has been suggested that these phenomena may be secondary to a defect in microtubule polymerization. We have examined the cytoskeleton of beige mouse cells by indirect immunofluorescence and find the number and distribution of microtubules and actin cables to be indistinguishable from those of normal control cells.
منابع مشابه
Chediak-Higashi syndrome is not due to a defect in microtubule-based lysosomal mobility.
Chediak-Higashi Syndrome is an autosomal recessive disorder that affects intracellular vesicle formation. The diagnostic feature of Chediak-Higashi Syndrome is the presence of 'giant' lysosomes clustered near the nucleus. Lysosome morphology in macrophages is maintained by microtubules and microtubule-based motors, such as kinesin. Dramatic changes in lysosome morphology can be induced by lower...
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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 a-D-mannosidase (pH 4.2) compared with normal controls. Additional studies revealed a small, but significant,...
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ورودعنوان ژورنال:
- The Journal of Cell Biology
دوره 79 شماره
صفحات -
تاریخ انتشار 1978