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The TCF-1 and LEF-1 Transcription Factors Have Cooperative and Opposing Roles in T Cell Development and Malignancy
Journal article   Open access   Peer reviewed

The TCF-1 and LEF-1 Transcription Factors Have Cooperative and Opposing Roles in T Cell Development and Malignancy

Shuyang Yu, Xinyuan Zhou, Farrah C Steinke, Chengyu Liu, Shann-Ching Chen, Oksana Zagorodna, Xuefang Jing, Yoshifumi Yokota, David K Meyerholz, Charles G Mullighan, …
Immunity (Cambridge, Mass.), Vol.37(5), pp.813-826
11/16/2012
DOI: 10.1016/j.immuni.2012.08.009
PMID: 23103132
url
https://doi.org/10.1016/j.immuni.2012.08.009View
Published (Version of record) Open Access

Abstract

The TCF-1 and LEF-1 transcription factors are known to play critical roles in normal thymocyte development. Unexpectedly, we found that TCF-1-deficient (Tcf7−/−) mice developed aggressive T cell malignancy, resembling human T cell acute lymphoblastic leukemia (T-ALL). LEF-1 was aberrantly upregulated in premalignant Tcf7−/− early thymocytes and lymphoma cells. We further demonstrated that TCF-1 directly repressed LEF-1 expression in early thymocytes and that conditional inactivation of Lef1 greatly delayed or prevented T cell malignancy in Tcf7−/− mice. In human T-ALLs, an early thymic progenitor (ETP) subtype was associated with diminished TCF7 expression, and two of the ETP-ALL cases harbored TCF7 gene deletions. We also showed that TCF-1 and LEF-1 were dispensable for T cell lineage commitment but instead were required for early thymocytes to mature beyond the CD4−CD8− stage. TCF-1 thus has dual roles, i.e., acting cooperatively with LEF-1 to promote thymocyte maturation while restraining LEF-1 expression to prevent malignant transformation of developing thymocytes. [Display omitted] ► TCF-1 and LEF-1 have both cooperative and opposing roles during T cell development ► TCF-1 and LEF-1 are required for β-selection but not T cell lineage commitment ► TCF-1 directly restrains LEF-1 expression to prevent thymocyte transformation ► ETP-ALLs are associated with decreased TCF1 expression and harbor TCF7 gene deletion

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