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Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b 558
Author(s) -
Mühlebach T. J.,
Erny C.,
Suter R.,
Brägger C.,
Yalman N.,
Kobayashi S.,
Nakamura M.,
Seger R. A.
Publication year - 1991
Publication title -
pediatric allergy and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.269
H-Index - 89
eISSN - 1399-3038
pISSN - 0905-6157
DOI - 10.1111/j.1399-3038.1991.tb00194.x
Subject(s) - chronic granulomatous disease , medicine , antibody , monoclonal antibody , cytochrome b , cytochrome , protein subunit , microbiology and biotechnology , cytochrome c , immunology , biology , gene , biochemistry , mitochondrial dna , mitochondrion , enzyme
Four different types of chronic granulomatous disease (CGD) were analysed with the monoclonal antibody, 7D5, directed against the small 23 kD subunit of cytochrome b 558 using a flow cytometric fluorescence analytical method. 7D5 immunofluorescent surface staining of granulocytes was absent in 12 patients with X‐linked cytochrome b 558 deficiency, in 2 patients with variant X‐linked CGD with residual (X‐forming activity and in 2 patients with autosomal recessive cytochrome b 558 deficiency. The mothers of the patients with the X‐linked form of CGD had 2 cell populations, one 7D5 negative or weakly positive and one 7D5 positive. The granulocytes of both parents of one patient with autosomal recessive cytochrome b 558 deficiency had slightly reduced fluorescence intensity comparable to their reduced cytochrome b 558 content. Three CGD patients with normal cytochrome b 558 and their parents had granulocytes normally stained with antibody 7D5. 7D5 antibody enables rapid detection and classification of CGD patients with cytochrome b 558 deficiency as well as rapid identification of heterozygous carriers.