B-cell lymphoma/leukemia 11B
Definition:
Key regulator of both differentiation and survival of T-lymphocytes during thymocyte development in mammals. Essential in controlling the responsiveness of hematopoietic stem cells to chemotactic signals by modulating the expression of the receptors CCR7 and CCR9, which direct the movement of progenitor cells from the bone marrow to the thymus. Is a regulator of IL2 promoter and enhances IL2 expression in activated CD4(+) T-lymphocytes. Tumor-suppressor that represses transcription through direct, TFCOUP2-independent binding to a GC-rich response element (By similarity). May also function in the P53-signaling pathway (By similarity).
References:
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[1]. Divya Punwani, et al. Multisystem Anomalies in Severe Combined Immunodeficiency with Mutant BCL11B. N Engl J Med. 2016 Dec 1;375(22):2165-2176. [Content Brief]
[2]. Valeriu B Cismasiu, et al. BCL11B participates in the activation of IL2 gene expression in CD4+ T lymphocytes. Blood. 2006 Oct 15;108(8):2695-702. [Content Brief]
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