IMPAIRED MICROTUBULE ASSEMBLY AND POLYMORPHONUCLEAR LEUKOCYTE FUNCTION IN THE CHEDIAK-HIGASHI-SYNDROME CORRECTABLE BY ASCORBIC-ACID
IMPAIRED MICROTUBULE ASSEMBLY AND POLYMORPHONUCLEAR LEUKOCYTE FUNCTION IN THE CHEDIAK-HIGASHI-SYNDROME CORRECTABLE BY ASCORBIC-ACID
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DOI：
10.1111/j.1365-2141.1979.tb03743.x
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发表时间：
1979-01-01
影响因子：
6.5
通讯作者：
OLIVER, JM
中科院分区：
文献类型：
--
作者：
BOXER, LA;ALBERTINI, DF;OLIVER, JM
Chediak-Higashi综合征（CHS）中多形核白细胞（PMN）的异常表面特征和杀菌功能缺陷先前与微管组装受损相关，在1例直接EM患者中，在表面膜被豆豆蛋白（Con） a激活后，微管组装异常。在另外2例正常PMN含有大量微管的情况下，CHS白细胞中可见很少的微管。体内和体外暴露于抗坏血酸的CHS白细胞促进了微管的组装。这种药物使CHS白细胞的趋化性和脱颗粒正常化，纠正了这些白细胞的粒细胞粘附性。抗坏血酸治疗后CHS患者临床病程的改善至少部分与抗坏血酸改善微管组装和PMN功能有关。
Abnormal surface characteristics and defective bactericidal function of polymorphonuclear leukocytes (PMN) in the Chediak-Higashi syndrome (CHS) were previously correlated with impaired microtubule assembly, and in 1 patient with direct EM evidence for an anomaly in microtubule assembly following surface membrane activation by concanavalin (Con) A. Very few microtubules were visible in CHS leukocytes from 2 additional patients under conditions where normal PMN contain abundant microtubules, and in vivo as well as in vitro exposure of the CHS leukocytes to ascorbic acid promoted the assembly of microtubules. This agent, which normalized chemotaxis and degranulation in CHS leukocytes, corrected granulocyte adherence in these leukocytes. The improved clinical course of patients with CHS following treatment with ascorbic acid is at least partly related to improvement of microtubule assembly and PMN function by the ascorbic acid.