Boi Michela, Zucca Emanuele, Inghirami Giorgio, Bertoni Francesco
Department of Pathology and Laboratory Medicine, Weill Cornell Medical College , New York, NY , USA.
Leuk Lymphoma. 2015 May;56(5):1223-8. doi: 10.3109/10428194.2014.953155. Epub 2014 Oct 7.
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.
编码人类BLIMP1的基因prdm1位于6号染色体的6q21区域,该位点在淋巴瘤中经常缺失。BLIMP1能够通过不同机制以依赖于上下文的方式使其靶基因沉默。BLIMP1在B细胞和T细胞中均有表达,并在其中发挥重要功能。在B细胞中,BLIMP1作为浆细胞分化的主要调节因子,受BCL6抑制,同时也抑制BCL6和PAX5。在T细胞中,BLIMP1是CD4+和CD8+T细胞中大多数终末效应细胞分化的关键因素。BLIMP1在多种淋巴瘤中经常失活,包括弥漫性大B细胞淋巴瘤、自然杀伤细胞淋巴瘤和间变性大T细胞淋巴瘤。在本综述中,我们将总结BLIMP1在正常细胞中的作用,重点是淋巴细胞,以及其在淋巴瘤中作为肿瘤抑制基因的功能。