Patients with CD3G mutations reveal a role for human CD3γ in Treg diversity and suppressive function-Reference-Cited by-同舟云学术

Patients with CD3G mutations reveal a role for human CD3γ in Treg diversity and suppressive function

Published:2018-05-24 Issue:21 Volume:131 Page:2335-2344
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Rowe Jared H.¹,Delmonte Ottavia M.²,Keles Sevgi³^ORCID,Stadinski Brian D.⁴^ORCID,Dobbs Adam K.²^ORCID,Henderson Lauren A.⁵^ORCID,Yamazaki Yasuhiro²^ORCID,Allende Luis M.⁶^ORCID,Bonilla Francisco A.⁵^ORCID,Gonzalez-Granado Luis I.⁷^ORCID,Celikbilek Celik Seyma³,Guner Sukru N.³^ORCID,Kapakli Hasan³,Yee Christina⁵^ORCID,Pai Sung-Yun¹⁸^ORCID,Huseby Eric S.⁴,Reisli Ismail³,Regueiro Jose R.⁹^ORCID,Notarangelo Luigi D.²^ORCID

Affiliation:

1. Division of Hematology-Oncology, Boston Children’s Hospital, Boston, MA;

2. Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD;

3. Division of Pediatric Immunology and Allergy, Meram Medical Faculty, Necmettin Erbakan University, Konya, Turkey;

4. Department of Pathology, University of Massachusetts Medical School, Worcester, MA;

5. Division of Immunology, Boston Children’s Hospital, Boston, MA;

6. Department of Immunology and

7. Primary Immunodeficiencies Unit, Department of Pediatrics, Hospital Universitario 12 de Octubre, Madrid, Spain;

8. Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA; and

9. Department of Immunology, Complutense University School of Medicine, imas12 Research Institute, Madrid, Spain

Abstract

Key Points CD3γ-deficient patients manifest T-cell phenotypic and functional defects that are especially prominent in Treg cells. The peripheral T-cell repertoire of CD3γ-deficient patients is restricted, with molecular signatures of self-reactivity.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/131/21/2335/1468756/blood835561.pdf

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4. Effect of CD3delta deficiency on maturation of alpha/beta and gamma/delta T-cell lineages in severe combined immunodeficiency;Dadi;N Engl J Med,2003

5. Severe combined immunodeficiency caused by deficiency in either the delta or the epsilon subunit of CD3;de Saint Basile;J Clin Invest,2004

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