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Description
TCL1 (T cell leukemia/lymphoma 1), MTCP1 (mature T cell proliferation 1) and TCL1b belong to the TCL1 proto-oncogene family. The TCL1 gene at chromosome 14q32.1 is co mmonly activated in T cell neoplasms by chromosome r earrangements such as inversions inv(14)(q11,q32) and translocations t(14,14)(q11,q32) or t(7,14)(q35,q32). TCL1 expression is limited to immature thymocytes in early T-cell progenitors and from pre-B to mature B cells in the B-cell lineage. The TCL1/MTCP1/TCL1b proto-oncogene activation is the hallmark of human T-cell prolymphocytic leukemia (T-PLL), a form of adult leukemia. In addition to T-PLL, TCL1 is overexpressed in Burkitt's lymphoma cell lines, the majority of AIDS-related non-Hodgkin's lymphoma-designated immunoblastic lymphoma plasmacytoids, lymphoblastic lymphoma, chronic lymphocytic leukemia, mantle cell lymphoma, follicular lymphoma, diffuse large B-cell lymphoma, and primary cutaneous B-cell lymphoma. It is also implicated in the development of hematopoietic abnormalities in patients with ataxia-telangiectasia. The members of the TCL1 proto-oncogene family bind to the serine/threonine kinase Akt (PKB), increasing its phosphorylation status and kinase activity. Akt is a crucial regulator in transduction of antiapoptotic and proliferative signals in T cells. TCL1 facilitates heterodimerization of different Akt molecules, which may contribute to the development of human malignancies associated w ith TCL1 overexpression.Synonyms: T-cell leukemia/lymphoma protein 1A, TCL-1