The NA "null" phenotype of a young man is caused by an FcγRIIIB gene deficiency while the products of the neighboring FcγRIIA and FcγRIIIA genes are present

被引:9
作者
Flesch, BK [1 ]
Achtert, G [1 ]
Bauer, F [1 ]
Neppert, J [1 ]
机构
[1] Univ Kiel, Inst Transfus Med, D-24105 Kiel, Germany
关键词
Fc gamma RIIIb deficiency; NA1/NA2; polymorphism; NA "null; SH antigen;
D O I
10.1007/s002770050392
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A 27-year-old man with an allergy to house dust mites was found to lack the Fc gamma RIIIb on his neutrophils, Cell surface marker and PCR techniques were used to investigate possible reasons for this deficiency. Agglutination and immunofluorescence assays using the man's neutrophils together with NA1- and NA2-specific antibodies were negative, and there was no reaction with the Fc gamma RIII-specific mAb 3G8. Indirect immunofluorescence demonstrated the presence of the CD24 molecule, which, like the Fc gamma RIIIb, is anchored to the cell membrane by glycosylphosphatidylinositol. Thus a lack of the Fc gamma RIIIb cell membrane anchor was excluded. PCR analysis confirmed the absence of the NA1- and NA2 alleles. The individual was therefore typed as NA"null". The products of those genes located together with the Fc gamma RIIIB gene within a complex on chromosome 1 (q23-24) were examined. Fc gamma RII was demonstrated on monocytes and B cells with the use of Fc gamma RII-specific monoclonal antibodies. About 5% of the individual's peripheral blood monocytes were positive with the 3G8 antibody, indicating the presence of Fc gamma RIIIa. From these data we concluded that the Fc gamma RIIIb deficiency on the neutrophil cell surface of this individual is due to a lack of the Fc gamma RIIIB gene while excluding a lack of the Fc gamma RIIA and the Fc gamma RIIIA genes.
引用
收藏
页码:215 / 220
页数:6
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