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

Jared H. Rowe,Ottavia M. Delmonte,Sevgi Keles,Brian D. Stadinski,Adam K. Dobbs,Lauren A. Henderson,Yasuhiro Yamazaki,Luis M. Allende,Francisco A. Bonilla,Luis I. Gonzalez-Granado,Seyma Celikbilek Celik,Sukru N. Guner,Hasan Kapakli,Christina Yee,Sung-Yun Pai,Eric S. Huseby,Ismail Reisli,Jose R. Regueiro,Luigi D. Notarangelo

Blood（2018）

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摘要

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.

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