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MYH9-RD患者中性粒细胞包涵体的定位和本质鉴定 被引量：2

Localization and identification of neutrophils inclusions in MYH9-RD patients
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摘要 目的检测和分析非肌性肌球蛋白重链ⅡA(NMMHC-ⅡA)在MYH9-RD患者中性粒细胞胞浆中的定位及表达,以确定包涵体的构成本质。方法应用间接免疫荧光技术,观察MYH9-RD患者及正常对照者NMMHC-ⅡA在胞浆中的定位和分布情况;应用Western blot技术检测和分析中性粒细胞中NMMHC-ⅡA在患者和正常对照者之间蛋白表达水平的差异。结果免疫荧光技术清楚地显示了患者中性粒细胞胞浆中呈绿色荧光的"梭形"包涵体的形态和轮廓,与瑞特姬姆萨染色显示的包涵体形状、大小基本一致,但更为清晰;而正常对照者除有散点状的荧光斑点外,无包涵体形成;Western blot结果显示患者NMMHC-ⅡA表达量较正常对照者降低。结论 MYH9-RD患者中性粒细胞包涵体的主要成份是NMMHC-ⅡA,NMMHC-ⅡA的显阴性作用导致了包涵体的形成。 To identify the formation characteristics of neutrophils inclusions of MYH9-RD patients,we detected and analyzed the localization and the expression of NMMHCⅡA in neutrophils of MYH9-RD patients.Indirect immunofluorescence technique and western blot were used to observe distribution of NMMHC-ⅡA and analyze NMMHC-ⅡA levels in neutrophils of MYH9-RD patients and normal family members.Spindle-like neutrophils inclusions with green fluorescence in MYH9-RD patients were markedly showed by immuno-fluorescence technique,which matched very well in localization,size and shape,compared with the inclusions displayed by Wright-Giemsa’s stain.Meanwhile,no inclusions in the normal family members were observed except a diffusive distribution of fluorescent spot in neutrophils cytoplasm.Furthermore,the result of Western blot showed that the expression of NMMHC-ⅡA in neutrophils of MYH9-RD patients was down regulated than normal family members.All the results indicate that the major component of neutrophils inclusions from this MYH9-RD family patients are NMMHC-ⅡA,and dominant negative effect of NMMHC-ⅡA is involved in the formation of inclusions.And immunofluorescence analysis is more sensitive and specific than Wright-Giemsa′s staining in detecting inclusions.
作者 张淑芳 王顺兰 张应爱 黄邓高
机构地区 中南大学湘雅医院附属海口医院中心实验室
出处 《免疫学杂志》 CAS CSCD 北大核心 2012年第2期104-107,共4页 Immunological Journal
关键词 MYH9-RD 中性粒细胞包涵体 荧光免疫测定 非肌性肌球蛋白重链ⅡA(NMMHC-ⅡA) MYH9-RD Neutrophils inclusions Fluoroimmunoassay NMMHC-11 A
分类号 R392.12 [医药卫生—免疫学] R446.113 [医药卫生—诊断学]
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参考文献19

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二级参考文献30

  • 1杨海燕,王兆钺,苏雁华,曹丽娟,白霞,阮长耿.一例Fechtner综合征临床与分子缺陷研究——附文献复习[J].中华血液学杂志,2007,28(3):160-164. 被引量：8
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  • 7Kunishima S, Matsushita T, Kojima T, et al. Immunofluorescence analysis of neutrophil nonmuscle myosin heavy chain-A in MYH9 disorders: association of subcellular localization with MYH9 mutations. Lab Invest, 2003, 83: 115-122.
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免疫学杂志
2012年 第2期

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