Evidence of innate lymphoid cell redundancy in humans

被引：254

作者：

Vely, Frederic ^{[1
,2
]}

Barlogis, Vincent ^{[3
]}

Vallentin, Blandine ^{[3
]}

Neven, Benedicte ^{[4
,5
,6
,7
]}

Piperoglou, Christelle ^{[1
,2
]}

Perchet, Thibaut ^{[8
,9
]}

Petit, Maxime ^{[8
,9
]}

Yessaad, Nadia ^{[10
]}

Touzot, Fabien ^{[5
,11
]}

Bruneau, Julie ^{[5
,12
]}

Mahlaoui, Nizar ^{[4
,6
,7
]}

Zucchini, Nicolas ^{[13
]}

Farnarier, Catherine ^{[2
]}

Michel, Gerard ^{[3
]}

Moshous, Despina ^{[4
,5
,6
,7
]}

Blanche, Stephane ^{[4
,5
,6
,7
]}

Dujardin, Arnaud ^{[14
]}

Spits, Hergen ^{[15
]}

Distler, Joerg H. W. ^{[16
]}

Ramming, Andreas ^{[16
]}

Picard, Capucine ^{[4
,5
,6
,7
,17
]}

Golub, Rachel ^{[8
,9
]}

Fischer, Alain ^{[4
,5
,6
,7
,18
]}

Vivier, Eric ^{[1
,2
]}

机构：

[1] Aix Marseille Univ, CNRS, INSERM, CIML, Marseille, France

[2] Hop Conception, APHM, Serv Immunol, Marseille, France

[3] Hop La Timone, APHM, Serv Hematol & Oncol Pediat, Marseille, France

[4] Hop Univ Necker Enfants Malades, AP HP, Ctr Reference Deficits Immunitaires Hereditaires, Paris, France

[5] Univ Paris 05, Sorbonne Paris Cite, Inst Imagine, Paris, France

[6] INSERM, Paris, France

[7] Univ Necker Enfants Malad, AP HP, Unite Immunol Hematol & Rhumatol Pediat, Paris, France

[8] INSERM, Inst Pasteur, Unite Lymphopoiese, Paris, France

[9] Univ Paris Diderot, Sorbonne Paris Cite, Cellule Pasteur, Paris, France

[10] Aix Marseille Univ, Ml mAbs Consortium, Marseille, France

[11] Hop Necker Enfants Malad, AP HP, Biotherapy Unit, Paris, France

[12] Hop Necker Enfants Malad, AP HP, Serv Anatomopathol, Paris, France

[13] BD Biosci, Le Pont De Claix, France

[14] Innate Pharma, Marseille, France

[15] Univ Amsterdam, Acad Med Ctr, Arizona Amsterdam, Netherlands

[16] Univ Erlangen Nurnberg, Dept Internal Med Rheumatol & Immunol, Erlangen, Germany

[17] Hop Necker Enfants Malad, AP HP, Study Ctr Immunodeficiencies, Paris, France

[18] Coll France, Paris, France

来源：

NATURE IMMUNOLOGY | 2016年 / 17卷 / 11期

基金：

欧洲研究理事会;

关键词：

SEVERE COMBINED IMMUNODEFICIENCY; DIFFERENTIATION IN-VIVO; SYSTEMIC-SCLEROSIS; SINGLE-CENTER; TRANSPLANTATION; DISEASE; INFLAMMATION; IMMUNITY; SUBSETS; RECONSTITUTION;

D O I：

10.1038/ni.3553

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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 gamma-chain cytokine receptor subunit IL-2R gamma 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.

引用

页码：1291 / 1299

页数：9

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