Inherited deficiency of stress granule ZNFX1 in patients with monocytosis and mycobacterial disease.


Journal

Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876

Informations de publication

Date de publication:
13 04 2021
Historique:
entrez: 20 4 2021
pubmed: 21 4 2021
medline: 27 11 2021
Statut: ppublish

Résumé

Human inborn errors of IFN-γ underlie mycobacterial disease, due to insufficient IFN-γ production by lymphoid cells, impaired myeloid cell responses to this cytokine, or both. We report four patients from two unrelated kindreds with intermittent monocytosis and mycobacterial disease, including bacillus Calmette-Guérin-osis and disseminated tuberculosis, and without any known inborn error of IFN-γ. The patients are homozygous for

Identifiants

pubmed: 33876776
pii: 2102804118
doi: 10.1073/pnas.2102804118
pmc: PMC8053974
pii:
doi:

Substances chimiques

Antigens, Neoplasm 0
ZNFX1 protein, human 0
Interferon-gamma 82115-62-6

Types de publication

Case Reports Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : NHGRI NIH HHS
ID : UM1 HG006504
Pays : United States
Organisme : NIAID NIH HHS
ID : R01 AI095983
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001866
Pays : United States
Organisme : NIH HHS
ID : S10 OD018521
Pays : United States
Organisme : NIAID NIH HHS
ID : R37 AI095983
Pays : United States
Organisme : NIAID NIH HHS
ID : R01 AI088364
Pays : United States
Organisme : NHGRI NIH HHS
ID : U24 HG008956
Pays : United States

Déclaration de conflit d'intérêts

The authors declare no competing interest.

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Auteurs

Tom Le Voyer (T)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Anna-Lena Neehus (AL)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Rui Yang (R)

St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Masato Ogishi (M)

St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Jérémie Rosain (J)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Fayhan Alroqi (F)

Immunology Division, Department of Pediatrics, King Abdulaziz Medical City, Riyadh 11426, Saudi Arabia.
King Abdullah International Medical Research Center, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia.

Maha Alshalan (M)

Genetics & Precision Medicine Department, King Abdullah Specialized Children's Hospital, King Abdulaziz Medical City, Riyadh 11426, Saudi Arabia.

Sophie Blumental (S)

Paediatric Infectious Diseases Unit, Queen Fabiola Children's University Hospital, 1020 Brussels, Belgium.

Fatima Al Ali (F)

Research Branch, Sidra Medicine, Doha, Qatar.

Taushif Khan (T)

Research Branch, Sidra Medicine, Doha, Qatar.

Manar Ata (M)

Research Branch, Sidra Medicine, Doha, Qatar.

Laurence Rozen (L)

Laboratory of Hematology LHUB-ULB, Free University of Brussels ULB, 1020 Brussels, Belgium.

Anne Demulder (A)

Laboratory of Hematology LHUB-ULB, Free University of Brussels ULB, 1020 Brussels, Belgium.

Paul Bastard (P)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Conor Gruber (C)

Department of Microbiology, Icahn School of Medicine at Mount Sinai, New York, NY 10029.
Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 10029.
The Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029.

Manon Roynard (M)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Yoann Seeleuthener (Y)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Franck Rapaport (F)

St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Benedetta Bigio (B)

St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Maya Chrabieh (M)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.

Danielle Sng (D)

Laboratory of Human Genetics and Therapeutics, Genome Institute of Singapore, Agency for Science, Technology and Research, 138648 Singapore.

Laureline Berteloot (L)

Pediatric Radiology Department, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, 75015 Paris, France.

Nathalie Boddaert (N)

Imagine Institute, University of Paris, 75015 Paris, France.
Pediatric Radiology Department, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, 75015 Paris, France.

Flore Rozenberg (F)

Virology Laboratory, Cochin Hospital, University of Paris, 75014 Paris, France.

Saleh Al-Muhsen (S)

Immunology Research Laboratory, College of Medicine, King Saud University, Riyadh 114362, Saudi Arabia.

Aida Bertoli-Avella (A)

Department of Genomic Research, CENTOGENE GmbH, 18055 Rostock, Germany.

Laurent Abel (L)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Dusan Bogunovic (D)

Laboratory of Hematology LHUB-ULB, Free University of Brussels ULB, 1020 Brussels, Belgium.
Department of Microbiology, Icahn School of Medicine at Mount Sinai, New York, NY 10029.
Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 10029.

Nico Marr (N)

Research Branch, Sidra Medicine, Doha, Qatar.
College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar.

Davood Mansouri (D)

Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran 19569-44413, Iran.
Department of Clinical Immunology and Infectious Diseases, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran 19569-44413, Iran.

Fuad Al Mutairi (F)

King Abdullah International Medical Research Center, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia.
Genetics & Precision Medicine Department, King Abdullah Specialized Children's Hospital, King Abdulaziz Medical City, Riyadh 11426, Saudi Arabia.

Vivien Béziat (V)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Dominique Weil (D)

Laboratory of Biology of Development, Institute of Biology Paris Seine (IBPS), CNRS, Sorbonne University, 75005, Paris, France.

Seyed Alireza Mahdaviani (SA)

Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran 19569-44413, Iran.

Alina Ferster (A)

Hemato-oncology Unit, Queen Fabiola Children's University Hospital, Brussels 1020, Belgium.

Shen-Ying Zhang (SY)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Bruno Reversade (B)

Laboratory of Human Genetics and Therapeutics, Genome Institute of Singapore, Agency for Science, Technology and Research, 138648 Singapore.

Stéphanie Boisson-Dupuis (S)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.

Jean-Laurent Casanova (JL)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France; jean-laurent.casanova@rockefeller.edu jacinta.bustamante@inserm.fr.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.
HHMI, New York, NY 10065.

Jacinta Bustamante (J)

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, 75015 Paris, France; jean-laurent.casanova@rockefeller.edu jacinta.bustamante@inserm.fr.
Imagine Institute, University of Paris, 75015 Paris, France.
St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065.
Study Center of Immunodeficiencies, Necker Hospital for Sick Children, 75015 Paris, France.

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