Chronic granulomatous disease with neutrophil membrane cytochrome b deficiency: Demonstration by immunochemical staining with monoclonal antibody.

Minegishi, Naoko; Nakamura, Michio; Suzaki, Kazuhiko; Terasawa, Masahiko; Minegishi, Masayoshi; Tsuchiya, Shigeru; Konno, Tasuke

doi:10.1620/tjem.154.143

Cited by 3 publications

(3 citation statements)

References 11 publications

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“…Complete X-linked-CGD Chronic granulomatous disease is a heterogeneous group of disorders with several molecular defects of different inheritance. The result of this study confirms earlier findings (19,20,17) that cytochrome b,,, is barely detectable or absent in a Triton X-100 extract and on the surface of granulocytes of most patients with X-linked CGD. It also confirms recent findings (8) that the light chain of cytochrome b,,, is not inserted into the membrane, when the heavy chain encoded on the X-chromosome is absent.…”

Section: Discussionsupporting

confidence: 92%

“…Nakamura et al (16) raised a monoclonal antibody, 7DS, against the small 23 kD subunit of cytochrome b,,, from human granulocytes, which allows the detection of the cytochrome b light chain on the surface of granulocytes by immunocytochemical analysis (17,18). With this antibody the absence of cytochrome b558 on the surface of granulocytes from patients with X-linked CGD was revealed (19,20) by flow cytometric analysis.…”

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confidence: 99%

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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 b₅₅₈

Mühlebach

Erny

Suter

et al. 1991

Pediatric Allergy Immunology

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Four different types of chronic granulomatous disease (CGD) were analysed with the monoclonal antibody, 7D5, directed against the small 23 kD subunit of cytochrome b558 using a flow cytometric fluorescence analytical method. 7D5 immunofluorescent surface staining of granulocytes was absent in 12 patients with X‐linked cytochrome b558 deficiency, in 2 patients with variant X‐linked CGD with residual (X‐forming activity and in 2 patients with autosomal recessive cytochrome b558 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 b558 deficiency had slightly reduced fluorescence intensity comparable to their reduced cytochrome b558 content. Three CGD patients with normal cytochrome b558 and their parents had granulocytes normally stained with antibody 7D5. 7D5 antibody enables rapid detection and classification of CGD patients with cytochrome b558 deficiency as well as rapid identification of heterozygous carriers.

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Section: Discussionsupporting

confidence: 92%

mentioning

confidence: 99%

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b₅₅₈

Mühlebach

Erny

Suter

et al. 1991

Pediatric Allergy Immunology

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“…As the heavy glycosylation of gp9l-phox makes quantification difficult, we used a polyclonal antibody raised against recombinant p22-phox. It has been well established previously that both subunits are mutually dependent for their stability [34][35][36][37][38] and so are present in equal amounts. The Western-blot analysis showed a striking reduction in the amount of cytochrome b558 in comparison with neutrophils and induced HL60 cells (Figure 4).…”

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confidence: 99%

Low NADPH oxidase activity in Epstein-Barr-virus-immortalized B-lymphocytes is due to a post-transcriptional block in expression of cytochrome b558

Chetty

Thrasher

Abo

et al. 1995

Biochemical Journal

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The NADPH oxidase of phagocytes is known to be expressed in Epstein-Barr-virus-transformed B-lymphocytes, albeit at levels only approx. 5% of those found in neutrophils. We have investigated the basis of this low level of expression and find that all four specific components of the NADPH oxidase are expressed in B-lymphocytes, but only p47-phox protein attains levels equivalent with those found in neutrophils. This component was shown to phosphorylate and translocate to the membrane normally on activation. The other cytosolic component, p67-phox, did show a deficit, and by supplementing a B-cell cytosol extract with recombinant p67-phox, this was shown to account for the somewhat reduced activity of B-cell cytosol in a cell-free oxidase system. The cell-free analysis also clearly located the major deficiency in superoxide-generating capacity of B-lymphocytes to the membrane. Western blotting of membrane proteins revealed major reductions in the amount of cytochrome b558. Analysis of the levels of mRNA for both subunits of cytochrome b558, however, showed levels greater than expected. Significantly more mRNA for gp91-phox was present in B-cells than in undifferentiated HL60 cells, although it was not quite as abundant as in differentiated HL60 cells, which are capable of producing large amounts of superoxide. We conclude that the failure of B-lymphocytes to generate amounts of superoxide equivalent to those generated by neutrophils is primarily due to a post-transcriptionally determined block to the accumulation of cytochrome b558.

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Severe phenotype of chronic granulomatous disease presenting in a female with a de novo mutation in gp91-phox and a non familial, extremely skewed X chromosome inactivation

Anderson-Cohen¹,

Holland²,

Kuhns

et al. 2003

Clinical Immunology

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Chronic granulomatous disease with neutrophil membrane cytochrome b deficiency: Demonstration by immunochemical staining with monoclonal antibody.

Cited by 3 publications

References 11 publications

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b₅₅₈

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b₅₅₈

Low NADPH oxidase activity in Epstein-Barr-virus-immortalized B-lymphocytes is due to a post-transcriptional block in expression of cytochrome b558

Severe phenotype of chronic granulomatous disease presenting in a female with a de novo mutation in gp91-phox and a non familial, extremely skewed X chromosome inactivation

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Chronic granulomatous disease with neutrophil membrane cytochrome b deficiency: Demonstration by immunochemical staining with monoclonal antibody.

Cited by 3 publications

References 11 publications

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b558

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b558

Low NADPH oxidase activity in Epstein-Barr-virus-immortalized B-lymphocytes is due to a post-transcriptional block in expression of cytochrome b558

Severe phenotype of chronic granulomatous disease presenting in a female with a de novo mutation in gp91-phox and a non familial, extremely skewed X chromosome inactivation

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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 b₅₅₈

Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b₅₅₈