期刊
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
卷 114, 期 3, 页码 459-466出版社
OXFORD UNIV PRESS INC
DOI: 10.1093/ajcp/114.3.459
关键词
paroxysmal nocturnal hemoglobinuria; aerolysin; GPI anchor proteins; CD59; aplastic anemia
类别
资金
- NCI NIH HHS [P50 CA058236, CA74990] Funding Source: Medline
Paroxysmal nocturnal hemoglobinuria (PNH) is caused by a somatic mutation in the gene PIGA which encodes an enzyme essential for the synthesis of glycosylphosphatidylinositol (GPI) anchors. The PIGA mutation results in absence or marked deficiency of more than a dozen proteins on PNH blood cells. Current flow cytometric assays for PNH rely on the use of labeled antibodies to detect deficiencies of specific GPI anchor proteins, such as CD59. However because no single GPI anchor protein is always expressed in all cell lineages, no one monoclonal antibody can be used with confidence to diagnose PNH. We describe a new diagnostic test for PNH, based on the ability of a fluorescently labeled inactive variant of the protein aerolysin (FLAER) to bind selectively to GPI anchors. We compared CPI anchor protein expression in 8 patients with PNH using FLAER and anti-CD59, In all cases, FLAER detected similar or higher proportions of PNH monocytes and granulocytes compared with anti-CD59. Because of the increased sensitivity of detection, FLAER could detect small abnormal granulocyte populations in patients to a level of about 0.5%; samples from healthy control subjects contained substantially fewer FLAER-negative cells. FLAER gives a more accurate assessment of the GPI anchor deficit in PNH.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据