The glycoprotein encoded by the X-linked chronic granulomatous disease locus is a component of the neutrophil cytochrome b complex
Abstract
The bacteriocidal capacity of phagocytic cells is impaired in X-linked chronic granulomatous disease (X-CGD), a disorder characterized by the absence of functional plasma-membrane-associated NADPH oxidase. The components of this oxidase system, their correspondence with specific genetic loci, and the primary protein defect in X-CGD remain incompletely defined. We recently reported cloning of the putative X-CGD gene on the basis of DNA linkage. To identify the predicted protein in vivo, antibodies were raised to a synthetic peptide derived from the complementary DNA sequence and to a fusion protein produced in Escherichia coli. In Western blots antisera detect a neutrophil protein of relative molecular mass in 90,000 (90K) that is absent in X-CGD patients. Antisera also react with the larger component of cytochrome b recently purified from neutrophil plasma membranes as a complex of glycosylated 90K and non-glycosylated 22K polypeptides. Based on our identification of the X-CGD protein in vivo, we propose that one of its critical roles is to interact with the 22K species to form a functional cytochrome b complex.
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H2O2 can modulate gene expression by epigenetic modification and activate transcription factors such as AP-1, NRF2, CREB, HSF1, HIF-1, TP53, NF-κβ, NOTCH, SP1, SCREB-1 and FOXO family.
“For example, H2O2 can also modulate genes expression by epigenetic modification and activate transcription factors such as AP-1, NRF2, CREB, HSF1, HIF-1, TP53, NF-κβ, NOTCH, SP1, SCREB-1 and FOXO family [14,15,16,17,18,19,20,21,22].”
From The Role of Reactive Species on Innate Immunity (2022) · cites this paper as [15]
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- Authors
- Dinauer, M. C., Orkin, S. H., Brown, R., Jesaitis, A. J., & Parkos, C. A.
- Affiliations
- Harvard University (US)
- Boston Children's Hospital (US)
- Scripps Clinic Medical Group (US)
- Venue
- Nature
- Published
- 1987-06
- Volume
- 327
- Issue
- 6124
- Pages
- 717-720
- ISSN
- 0028-0836 (ISSN-L)
- 1476-4687
- OpenAlex
- W2034290678
- Cited by
- 407
- References
- 19
- Citations by year
- 2026: 5 · 2025: 6 · 2024: 4 · 2023: 8 · 2022: 6 · 2021: 6 · 2020: 6 · 2019: 10 · 2018: 10 · 2017: 3 · 2016: 10 · 2015: 7 · 2014: 12 · 2013: 5 · 2012: 5
- FWCI
- 14.75
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Redox biology and oxidative stress
- Keywords
- X-linked chronic granulomatous disease, NADPH oxidase, glycoproteins, 22 kDa protein, gene cloning, genetic linkage
- MeSH terms
- Antigens, Cytochrome b Group, DNA, DNA, Recombinant, Glycoproteins, Granulomatous Disease, Chronic, Humans, Immune Sera, Immunoassay, Neutrophils, Recombinant Fusion Proteins, Recombinant Proteins, X Chromosome
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