SUPEROXIDE PRODUCTION BY NORMAL AND CHRONIC GRANULOMATOUS-DISEASE (CGD) PATIENT-DERIVED EBV-TRANSFORMED B-CELL LINES MEASURED BY CHEMILUMINESCENCE-BASED ASSAYS

被引：31

作者：

PORTER, CD ^{[1
]}

PARKAR, MH ^{[1
]}

COLLINS, MKL ^{[1
]}

LEVINSKY, RJ ^{[1
]}

KINNON, C ^{[1
]}

机构：

[1] INST CHILD HLTH,CHESTER BEATTY LABS,LONDON WC1N 1EH,ENGLAND

来源：

JOURNAL OF IMMUNOLOGICAL METHODS | 1992年 / 155卷 / 02期

关键词：

NADPH OXIDASE; CHEMILUMINESCENCE; CHRONIC GRANULOMATOUS DISEASE;

D O I：

10.1016/0022-1759(92)90281-W

中图分类号：

Q5 [生物化学];

学科分类号：

071010 ; 081704 ;

摘要：

We have compared assays for products of the neutrophil respiratory burst in normal EBV-transformed B cell lines stimulated with agonists of protein kinase C. Those measuring O2- directly or its immediate product, H2O2, were successful. Of these, the most sensitive were the lucigenin- and luminol-based chemiluminescence assays for O2- and H2O2 respectively. Cell lines from CGD patients, with X-linked or autosomal recessive genetic defects in the neutrophil NADPH oxidase, did not respond in these assays, indicative of their inability to produce O2-. The defects in the lines studied encompass both proteins forming the cytochrome b-245 membrane component, and the 47 kDa cytosolic component of the NADPH oxidase. The possession of the disease associated phenotype by these cell lines provides evidence that in the normal situation both neutrophils and B cells produce O2- via the same system.

引用

页码：151 / 157

页数：7