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Nat Immunol. 2016 Nov;17(11):1291-1299. doi: 10.1038/ni.3553. Epub 2016 Sep 12.

Evidence of innate lymphoid cell redundancy in humans.

Vély F1,2, Barlogis V3,4, Vallentin B3, Neven B4,5,6,7, Piperoglou C1,2, Ebbo M1,8, Perchet T9,10, Petit M9,10, Yessaad N11, Touzot F5,12, Bruneau J5,13, Mahlaoui N4,5,6,7, Zucchini N14, Farnarier C2, Michel G3, Moshous D4,5,6,7, Blanche S4,5,6,7, Dujardin A15, Spits H16, Distler JH17, Ramming A17, Picard C4,5,6,7,18, Golub R9,10, Fischer A4,5,6,7,19, Vivier E1,2.

Author information

1
Aix Marseille Université, CNRS, INSERM, CIML, Marseille, France.
2
APHM, Hôpital de la Conception, Service d'Immunologie, Marseille, France.
3
APHM, Hôpital de la Timone, Service d'Hématologie et Oncologie Pédiatrique, Marseille, France.
4
APHP, Hôpital Universitaire Necker-Enfants Malades, Centre de Référence Déficits Immunitaires Héréditaires, Paris, France.
5
Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine, Paris, France.
6
INSERM, Paris, France.
7
APHP, Hôpital Universitaire Necker-Enfants Malades, Unité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, Paris, France.
8
APHM, Hôpital de la Timone, Service de Médecine Interne, Marseille, France.
9
Institut Pasteur, Unité de Lymphopoièse, INSERM, Paris, France.
10
Université Paris Diderot, Sorbonne Paris Cité, Cellule Pasteur, Paris, France.
11
MI-mAbs consortium, Aix-Marseille University, Marseille, France.
12
APHP, Hôpital Necker-Enfants Malades, Biotherapy Unit, Paris, France.
13
APHP, Hôpital Necker-Enfants Malades, Service d'anatomopathologie, Paris, France.
14
BD Biosciences, Le Pont-de-Claix, France.
15
Innate-Pharma, Marseille, France.
16
Academic Medical Center at the University of Amsterdam, Arizona Amsterdam, the Netherlands.
17
Department of Internal Medicine, Rheumatology &Immunology, University of Erlangen-Nuremberg, Erlangen, Germany.
18
APHP, Hôpital Necker-Enfants Malades, Study Center of Immunodeficiencies, Paris, France.
19
College de France, Paris, France.

Abstract

Innate lymphoid cells (ILCs) have potent immunological functions in experimental conditions in mice, but their contributions to immunity in natural conditions in humans have remained unclear. We investigated the presence of ILCs in a cohort of patients with severe combined immunodeficiency (SCID). All ILC subsets were absent in patients with SCID who had mutation of the gene encoding the common γ-chain cytokine receptor subunit IL-2Rγ or the gene encoding the tyrosine kinase JAK3. T cell reconstitution was observed in patients with SCID after hematopoietic stem cell transplantation (HSCT), but the patients still had considerably fewer ILCs in the absence of myeloablation than did healthy control subjects, with the exception of rare cases of reconstitution of the ILC1 subset of ILCs. Notably, the ILC deficiencies observed were not associated with any particular susceptibility to disease, with follow-up extending from 7 years to 39 years after HSCT. We thus report here selective ILC deficiency in humans and show that ILCs might be dispensable in natural conditions, if T cells are present and B cell function is preserved.

PMID:
27618553
PMCID:
PMC5074366
DOI:
10.1038/ni.3553
[Indexed for MEDLINE]
Free PMC Article

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