Frontiers in Immunology (Jul 2021)
Genetic Screening for TLR7 Variants in Young and Previously Healthy Men With Severe COVID-19
- Xavier Solanich,
- Gardenia Vargas-Parra,
- Gardenia Vargas-Parra,
- Caspar I. van der Made,
- Caspar I. van der Made,
- Caspar I. van der Made,
- Annet Simons,
- Janneke Schuurs-Hoeijmakers,
- Arnau Antolí,
- Jesús del Valle,
- Jesús del Valle,
- Gemma Rocamora-Blanch,
- Fernando Setién,
- Manel Esteller,
- Manel Esteller,
- Manel Esteller,
- Manel Esteller,
- Simon V. van Reijmersdal,
- Antoni Riera-Mestre,
- Antoni Riera-Mestre,
- Joan Sabater-Riera,
- Gabriel Capellá,
- Gabriel Capellá,
- Frank L. van de Veerdonk,
- Frank L. van de Veerdonk,
- Ben van der Hoven,
- Xavier Corbella,
- Xavier Corbella,
- Alexander Hoischen,
- Alexander Hoischen,
- Alexander Hoischen,
- Conxi Lázaro,
- Conxi Lázaro
Affiliations
- Xavier Solanich
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Gardenia Vargas-Parra
- Hereditary Cancer Program, Catalan Institute of Oncology, Program in Molecular Mechanisms and Experimental Therapy in Oncology (Oncobell), Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Gardenia Vargas-Parra
- Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
- Caspar I. van der Made
- Department of Human Genetics, Radboud University Medical Center, Nijmegen, Netherlands
- Caspar I. van der Made
- Department of Internal Medicine and Radboud Center for Infectious Diseases (RCI), Radboud Institute for Molecular Life Sciences (RIMLS), Radboud University Medical Center, Nijmegen, Netherlands
- Caspar I. van der Made
- Radboud Expertise Center for Immunodeficiency and Autoinflammation and Radboud Center for Infectious Disease (RCI), Radboud University Medical Center, Nijmegen, Netherlands
- Annet Simons
- Department of Human Genetics, Radboud University Medical Center, Nijmegen, Netherlands
- Janneke Schuurs-Hoeijmakers
- Department of Human Genetics, Radboud University Medical Center, Nijmegen, Netherlands
- Arnau Antolí
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Jesús del Valle
- Hereditary Cancer Program, Catalan Institute of Oncology, Program in Molecular Mechanisms and Experimental Therapy in Oncology (Oncobell), Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Jesús del Valle
- Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
- Gemma Rocamora-Blanch
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Fernando Setién
- Josep Carreras Leukaemia Research Institute (IJC), Badalona, Spain
- Manel Esteller
- Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
- Manel Esteller
- Josep Carreras Leukaemia Research Institute (IJC), Badalona, Spain
- Manel Esteller
- Institucio Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain
- Manel Esteller
- Physiological Sciences Department, School of Medicine and Health Sciences, University of Barcelona (UB), Barcelona, Spain
- Simon V. van Reijmersdal
- Department of Human Genetics, Radboud University Medical Center, Nijmegen, Netherlands
- Antoni Riera-Mestre
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Antoni Riera-Mestre
- 0Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain
- Joan Sabater-Riera
- 1Department of Intensive Care, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Gabriel Capellá
- Hereditary Cancer Program, Catalan Institute of Oncology, Program in Molecular Mechanisms and Experimental Therapy in Oncology (Oncobell), Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Gabriel Capellá
- Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
- Frank L. van de Veerdonk
- Department of Internal Medicine and Radboud Center for Infectious Diseases (RCI), Radboud Institute for Molecular Life Sciences (RIMLS), Radboud University Medical Center, Nijmegen, Netherlands
- Frank L. van de Veerdonk
- Radboud Expertise Center for Immunodeficiency and Autoinflammation and Radboud Center for Infectious Disease (RCI), Radboud University Medical Center, Nijmegen, Netherlands
- Ben van der Hoven
- 2Department of Intensive Care, Erasmus MC, Rotterdam, Netherlands
- Xavier Corbella
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Xavier Corbella
- 3School of Medicine, Universitat Internacional de Catalunya, Barcelona, Spain
- Alexander Hoischen
- Department of Human Genetics, Radboud University Medical Center, Nijmegen, Netherlands
- Alexander Hoischen
- Department of Internal Medicine and Radboud Center for Infectious Diseases (RCI), Radboud Institute for Molecular Life Sciences (RIMLS), Radboud University Medical Center, Nijmegen, Netherlands
- Alexander Hoischen
- Radboud Expertise Center for Immunodeficiency and Autoinflammation and Radboud Center for Infectious Disease (RCI), Radboud University Medical Center, Nijmegen, Netherlands
- Conxi Lázaro
- Hereditary Cancer Program, Catalan Institute of Oncology, Program in Molecular Mechanisms and Experimental Therapy in Oncology (Oncobell), Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
- Conxi Lázaro
- Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
- DOI
- https://doi.org/10.3389/fimmu.2021.719115
- Journal volume & issue
-
Vol. 12
Abstract
IntroductionLoss-of-function TLR7 variants have been recently reported in a small number of males to underlie strong predisposition to severe COVID-19. We aimed to determine the presence of these rare variants in young men with severe COVID-19.MethodsWe prospectively studied males between 18 and 50 years-old without predisposing comorbidities that required at least high-flow nasal oxygen to treat COVID-19. The coding region of TLR7 was sequenced to assess the presence of potentially deleterious variants.ResultsTLR7 missense variants were identified in two out of 14 patients (14.3%). Overall, the median age was 38 (IQR 30-45) years. Both variants were not previously reported in population control databases and were predicted to be damaging by in silico predictors. In a 30-year-old patient a maternally inherited variant [c.644A>G; p.(Asn215Ser)] was identified, co-segregating in his 27-year-old brother who also contracted severe COVID-19. A second variant [c.2797T>C; p.(Trp933Arg)] was found in a 28-year-old patient, co-segregating in his 24-year-old brother who developed mild COVID-19. Functional testing of this variant revealed decreased type I and II interferon responses in peripheral mononuclear blood cells upon stimulation with the TLR7 agonist imiquimod, confirming a loss-of-function effect.ConclusionsThis study supports a rationale for the genetic screening for TLR7 variants in young men with severe COVID-19 in the absence of other relevant risk factors. A diagnosis of TLR7 deficiency could not only inform on treatment options for the patient, but also enables pre-symptomatic testing of at-risk male relatives with the possibility of instituting early preventive and therapeutic interventions.
Keywords