title: PRDM1/BLIMP1: a tumor suppressor gene in B and T cell lymphomas.
abstract: The gene encoding the human BLIMP1, prdm1, is located on chromosome 6q21, a locus frequently deleted in lymphoid tumors. BLIMP1 is able to silence its target genes in a context-dependent manner through different mechanisms. BLIMP1 is expressed in both B and T cells, in which it plays important functions. In B cells, BLIMP1 acts as the master regulator of plasma cell differentiation, repressed by BCL6 and repressing both BCL6 and PAX5. In T cells, BLIMP1 is a critical factor for most terminal effector cell differentiation in both CD4+ and CD8+ T cells. BLIMP1 is frequently inactivated in a variety of lymphomas, including diffuse large B cell lymphomas, Natural Killer cell lymphoma and anaplastic large T cell lymphoma. In this review, we will summarize the role of BLIMP1 in normal cells, focusing on lymphoid cells, and on its function as tumor suppressor gene in lymphomas.
authors.affiliation: Department of Pathology and Laboratory Medicine, Weill Cornell Medical College , New York, NY , USA.
authors.forename: Michela
authors.initials: M
authors.lastname: Boi
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Giorgio
authors.initials: G
authors.lastname: Inghirami
authors.forename: Francesco
authors.initials: F
authors.lastname: Bertoni
mesh_terms: D000818:Animals; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000378:metabolism; D006801:Humans; D016393:Lymphoma, B-Cell / Q000235:genetics* / Q000378:metabolism; D016399:Lymphoma, T-Cell / Q000235:genetics* / Q000378:metabolism; D000074462:Positive Regulatory Domain I-Binding Factor 1; D012097:Repressor Proteins / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D025521:Tumor Suppressor Proteins / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism
chemical_list: D012097:Repressor Proteins; D025521:Tumor Suppressor Proteins; C087042:PRDM1 protein, human; D000074462:Positive Regulatory Domain I-Binding Factor 1
keywords: ALCL; BCL6; BLIMP1; DLBCL; NK; PAX5; PRDM1