Modeling altered T-cell development with induced pluripotent stem cells from patients with RAG1-dependent immune deficiencies.

doi:10.1182/blood-2015-10-676304

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Modeling altered T-cell development with induced pluripotent stem cells from patients with RAG1-dependent immune deficiencies.

Blood - 11 Aug 2016

Brauer Patrick M, Pessach Itai M, Clarke Erik, Rowe Jared H, Ott de Bruin Lisa, Lee Yu Nee, Dominguez-Brauer Carmen, Comeau Anne M, Awong Geneve, Felgentreff Kerstin, Zhang Yuhang H, Bredemeyer Andrea, Al-Herz Waleed, Du Likun, Ververs Francesca, Kennedy Marion, Giliani Silvia, Keller Gordon, Sleckman Barry P, Schatz David G, Bushman Frederic D, Notarangelo Luigi D, Zúñiga-Pflücker Juan Carlos

Abstract excerpt

Primary immunodeficiency diseases comprise a group of heterogeneous genetic defects that affect immune system development and/or function. Here we use in vitro differentiation of human induced pluripotent stem cells (iPSCs) generated from patients with different recombination-activating gene 1 (RAG1) mutations to assess T-cell development and T-cell receptor (TCR) V(D)J recombination. RAG1-mutants from severe...

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Identifiers

PMID: 27301863
DOI: 10.1182/blood-2015-10-676304
PMCID: PMC4982452

Genes

RAG1
TRA
TRB

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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