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The importance of the system in host defence and immunoregulation is underlined by chronic granulomatous disease (CGD), a severe monogenic immunodeficiency caused by mutations in genes encoding individual components of NOX2. CGD also leads to inflammatory manifestations due to the regulatory role of ROS in multiple signalling processes. We describe the system in detail, from its discovery to our current understanding of the oxidase. We also describe CGD and illustrate how recent insights into this disease shed light on physiology.<\/jats:p>","DOI":"10.1093\/cei\/uxaf031","type":"journal-article","created":{"date-parts":[[2025,5,12]],"date-time":"2025-05-12T07:48:15Z","timestamp":1747036095000},"source":"Crossref","is-referenced-by-count":9,"title":["Chronic granulomatous disease: lessons in cell biology from monogenic immunodeficiency"],"prefix":"10.1093","volume":"219","author":[{"given":"Paige M","family":"Mortimer","sequence":"first","affiliation":[{"name":"Centre for Inflammatory Disease, Department of Immunology & Inflammation, Imperial College , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuli","family":"Svetitsky","sequence":"additional","affiliation":[{"name":"The Francis Crick Institute , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David C","family":"Thomas","sequence":"additional","affiliation":[{"name":"Cambridge Institute for Therapeutic Immunology and Infectious Disease University of Cambridge ,","place":["UK"]},{"name":"Department of Medicine, University of Cambridge ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,5,15]]},"reference":[{"key":"2025112708214073000_CIT0001","doi-asserted-by":"publisher","first-page":"104","DOI":"10.1186\/s12974-015-0325-5","article-title":"Inhibition of NADPH oxidase activation reduces EAE-induced white matter damage in mice","volume":"12","author":"Choi","year":"2015","journal-title":"J Neuroinflammation"},{"key":"2025112708214073000_CIT0002","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1002\/ana.410230505","article-title":"Suppression of experimental autoimmune neuritis by the oxygen radical scavengers superoxide dismutase and catalase","volume":"23","author":"Hartung","year":"1988","journal-title":"Ann Neurol"},{"key":"2025112708214073000_CIT0003","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1038\/nature13152","article-title":"NRROS negatively regulates reactive oxygen species during host defence and autoimmunity","volume":"509","author":"Noubade","year":"2014","journal-title":"Nature"},{"key":"2025112708214073000_CIT0004","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1152\/AJPLEGACY.1932.103.1.235","article-title":"The extra respiration of phagocytosis","volume":"103","author":"Baldridge","year":"1932","journal-title":"Am J Physiol"},{"key":"2025112708214073000_CIT0005","doi-asserted-by":"publisher","first-page":"2224","DOI":"10.1016\/s0021-9258(18)64602-2","article-title":"The biochemical basis of phagocytosis","volume":"235","author":"Sbarra","year":"1959","journal-title":"J Biol Chem"},{"key":"2025112708214073000_CIT0006","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1038\/192535a0","article-title":"Biochemical aspects of phagocytosis","volume":"192","author":"IYER","year":"1961","journal-title":"Nature"},{"key":"2025112708214073000_CIT0007","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1016\/0304-4165(68)90116-5","article-title":"The role of the phagocyte in host-parasite interactions","volume":"156","author":"Paul","year":"1968","journal-title":"Biochimica et Biophysica Acta (BBA) - General Subjects"},{"key":"2025112708214073000_CIT0008","doi-asserted-by":"publisher","first-page":"945","DOI":"10.1172\/JCI108024","article-title":"H2O2 release from human granulocytes during phagocytosis. 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