UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Impaired respiratory burst contributes to infections in PKCδ-deficient patients

Neehus, A-L; Moriya, K; Nieto-Patlán, A; Le Voyer, T; Lévy, R; Özen, A; Karakoc-Aydiner, E; ... Bustamante, J; + view all (2021) Impaired respiratory burst contributes to infections in PKCδ-deficient patients. Journal of Experimental Medicine , 218 (9) , Article e20210501. 10.1084/jem.20210501. Green open access

[thumbnail of jem_20210501.pdf]
Preview
Text
jem_20210501.pdf - Published Version

Download (7MB) | Preview

Abstract

Patients with autosomal recessive protein kinase C δ (PKCδ) 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 PKCδ-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 PKCδ in activation of the NADPH oxidase complex. Our findings thus show that patients with PKCδ deficiency have impaired NADPH oxidase activity in various myeloid subsets, which may contribute to their CGD-like infectious phenotype.

Type: Article
Title: Impaired respiratory burst contributes to infections in PKCδ-deficient patients
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1084/jem.20210501
Publisher version: http://dx.doi.org/10.1084/jem.20210501
Language: English
Additional information: © 2021 Neehus et al. This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms/). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 International license, as described at https://creativecommons.org/licenses/by-nc-sa/4.0/).
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10131996
Downloads since deposit
267Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item