{"id":64057,"date":"2023-01-19T16:07:36","date_gmt":"2023-01-19T15:07:36","guid":{"rendered":"https:\/\/cri1149.fr\/?post_type=publications&#038;p=64057"},"modified":"2025-09-11T16:47:58","modified_gmt":"2025-09-11T14:47:58","slug":"impaired-respiratory-burst-contributes-to-infections-in-pkc%ce%b4-deficient-patients","status":"publish","type":"publication","link":"https:\/\/cri1149.fr\/en\/publication\/impaired-respiratory-burst-contributes-to-infections-in-pkc%ce%b4-deficient-patients\/","title":{"rendered":"Impaired respiratory burst contributes to infections in PKC\u03b4-deficient patients"},"content":{"rendered":"","protected":false},"featured_media":0,"template":"","meta":{"_acf_changed":false,"_EventAllDay":false,"_EventTimezone":"","_EventStartDate":"","_EventEndDate":"","_EventStartDateUTC":"","_EventEndDateUTC":"","_EventShowMap":false,"_EventShowMapLink":false,"_EventURL":"","_EventCost":"","_EventCostDescription":"","_EventCurrencySymbol":"","_EventCurrencyCode":"","_EventCurrencyPosition":"","_EventDateTimeSeparator":"","_EventTimeRangeSeparator":"","_EventOrganizerID":[],"_EventVenueID":[],"_OrganizerEmail":"","_OrganizerPhone":"","_OrganizerWebsite":"","_VenueAddress":"","_VenueCity":"","_VenueCountry":"","_VenueProvince":"","_VenueState":"","_VenueZip":"","_VenuePhone":"","_VenueURL":"","_VenueStateProvince":"","_VenueLat":"","_VenueLng":"","_VenueShowMap":false,"_VenueShowMapLink":false},"category_publication":[],"class_list":["post-64057","publication","type-publication","status-publish","hentry"],"acf":{"numero_de_publication":"J Exp Med.","date_de_publication":"20210906","numero_doi":"","equipe":[133],"auteurs-liste":[{"texte_libre":true,"auteur-lien":null,"auteur-text":"Anna-Lena Neehus"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Kunihiko Moriya"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Alejandro Nieto-Patl\u00e1n"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Tom Le Voyer"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Romain L\u00e9vy"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Ahmet \u00d6zen"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Elif Karakoc-Aydiner"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Safa Baris"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Alisan Yildiran"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Engin Altundag"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Manon Roynard"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Kathrin Haake"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"M\u00e9lanie Migaud"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Karim Dorgham"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Guy Gorochov"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Laurent Abel"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Nico Lachmann"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Figen Dogu"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Sule Haskologlu"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Erdal \u0130nce"},{"texte_libre":false,"auteur-lien":13071,"auteur-text":""},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Gulbu Uzel"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Ayca Kiykim"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Kaan Boztug"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Marion R Roderick"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Mohammad Shahrooei"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Paul A Brogan"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Hassan Abolhassani"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Gonca Hancioglu"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Nima Parvaneh"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Alexandre Belot"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Aydan Ikinciogullari "},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Jean-Laurent Casanova"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Anne Puel"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Jacinta Bustamante"}],"auteurs-manuel":"","liens_externes":null,"liens":[{"lien":"https:\/\/pubmed.ncbi.nlm.nih.gov\/34264265\/"}],"paragraphe":"[:en]<h2 class=\"title\" style=\"text-align: justify;\">Abstract<\/h2>\r\n<div id=\"eng-abstract\" class=\"abstract-content selected\">\r\n<p style=\"text-align: justify;\">Patients with autosomal recessive protein kinase C \u03b4 (PKC\u03b4) deficiency suffer from childhood-onset autoimmunity, including systemic lupus erythematosus. They also suffer from recurrent infections that overlap with those seen in patients with chronic granulomatous disease (CGD), a disease caused by defects of the phagocyte NADPH oxidase and a lack of reactive oxygen species (ROS) production. We studied an international cohort of 17 PKC\u03b4-deficient patients and found that their EBV-B cells and monocyte-derived phagocytes produced only small amounts of ROS and did not phosphorylate p40phox normally after PMA or opsonized Staphylococcus aureus stimulation. Moreover, the patients' circulating phagocytes displayed abnormally low levels of ROS production and markedly reduced neutrophil extracellular trap formation, altogether suggesting a role for PKC\u03b4 in activation of the NADPH oxidase complex. Our findings thus show that patients with PKC\u03b4 deficiency have impaired NADPH oxidase activity in various myeloid subsets, which may contribute to their CGD-like infectious phenotype.<\/p>\r\n<\/div>[:]","paragraphe_en":"[:en]<h2 class=\"title\" style=\"text-align: justify;\">Abstract<\/h2>\r\n<div id=\"eng-abstract\" class=\"abstract-content selected\">\r\n<p style=\"text-align: justify;\">Patients with autosomal recessive protein kinase C \u03b4 (PKC\u03b4) deficiency suffer from childhood-onset autoimmunity, including systemic lupus erythematosus. They also suffer from recurrent infections that overlap with those seen in patients with chronic granulomatous disease (CGD), a disease caused by defects of the phagocyte NADPH oxidase and a lack of reactive oxygen species (ROS) production. We studied an international cohort of 17 PKC\u03b4-deficient patients and found that their EBV-B cells and monocyte-derived phagocytes produced only small amounts of ROS and did not phosphorylate p40phox normally after PMA or opsonized Staphylococcus aureus stimulation. Moreover, the patients' circulating phagocytes displayed abnormally low levels of ROS production and markedly reduced neutrophil extracellular trap formation, altogether suggesting a role for PKC\u03b4 in activation of the NADPH oxidase complex. Our findings thus show that patients with PKC\u03b4 deficiency have impaired NADPH oxidase activity in various myeloid subsets, which may contribute to their CGD-like infectious phenotype.<\/p>\r\n<\/div>[:]","documents":null},"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Impaired respiratory burst contributes to infections in PKC\u03b4-deficient patients - CRI<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/cri1149.fr\/publication\/impaired-respiratory-burst-contributes-to-infections-in-pkc\u03b4-deficient-patients\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Impaired respiratory burst contributes to infections in PKC\u03b4-deficient patients - CRI\" \/>\n<meta property=\"og:url\" content=\"https:\/\/cri1149.fr\/publication\/impaired-respiratory-burst-contributes-to-infections-in-pkc\u03b4-deficient-patients\/\" \/>\n<meta property=\"og:site_name\" content=\"CRI\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/CRI-Centre-de-recherche-sur-linflammation-100116118884153\" \/>\n<meta property=\"article:modified_time\" content=\"2025-09-11T14:47:58+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/cri1149.fr\\\/publication\\\/impaired-respiratory-burst-contributes-to-infections-in-pkc%ce%b4-deficient-patients\\\/\",\"url\":\"https:\\\/\\\/cri1149.fr\\\/publication\\\/impaired-respiratory-burst-contributes-to-infections-in-pkc%ce%b4-deficient-patients\\\/\",\"name\":\"Impaired respiratory burst contributes to infections in PKC\u03b4-deficient patients - 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