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Impaired microtubule assembly and polymorphonuclear leucocyte function in the Chediak-Higashi syndrome correctable by ascorbic acid.

Abstract：

:It was previously shown that the abnormal surface characteristics and defective bactericidal function of polymorphonuclear leucocytes (PMN) in the Chediak-Higashi syndrome (CHS) are correlated with impaired microtubule assembly, and in one patient direct electron microscopic evidence for an anomaly in microtubule assembly following surface membrane activation by concanavalin A (Con A). We show that very few microtubules are visible in CHS leucocytes from two additional patients under conditions where normal PMNs contain abundant microtubules, and that both in vivo and in vitro exposure of the CHS leucocytes to ascorbic acid promotes the assembly of microtubules. This agent, which normalizes chemotaxis and degranulation in CHS leucocytes, is shown also to correct granulocyte adherence in these leucocytes. It is suggested that the improved clinical course of patients with CHS following treatment with ascorbic acid is related at least in part to improvement of microtubule assembly and PMN function by the ascorbic acid.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

Boxer LA,Albertini DF,Baehner RL,Oliver JM

doi

10.1111/j.1365-2141.1979.tb03743.x

subject

Has Abstract

pub_date

1979-10-01 00:00:00

pages

207-13

issue

2

eissn

0007-1048

issn

1365-2141

journal_volume

43

pub_type

杂志文章

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