| First Author | Mandelbaum J | Year | 2010 |
| Journal | Cancer Cell | Volume | 18 |
| Issue | 6 | Pages | 568-79 |
| PubMed ID | 21156281 | Mgi Jnum | J:167435 |
| Mgi Id | MGI:4868289 | Doi | 10.1016/j.ccr.2010.10.030 |
| Citation | Mandelbaum J, et al. (2010) BLIMP1 is a tumor suppressor gene frequently disrupted in activated B cell-like diffuse large B cell lymphoma. Cancer Cell 18(6):568-79 |
| abstractText | Diffuse large B cell lymphoma (DLBCL) is a heterogeneous disease composed of at least two distinct subtypes: germinal center B cell-like (GCB) and activated B cell-like (ABC) DLBCL. These phenotypic subtypes segregate with largely unique genetic lesions, suggesting the involvement of different pathogenetic mechanisms. In this report we show that the BLIMP1/PRDM1 gene is inactivated by multiple mechanisms, including homozygous deletions, truncating or missense mutations, and transcriptional repression by constitutively active BCL6, in approximately 53% of ABC-DLBCL. In vivo, conditional deletion of Blimp1 in mouse B cells promotes the development of lymphoproliferative disorders recapitulating critical features of the human ABC-DLBCL. These results demonstrate that BLIMP1 is a bona fide tumor-suppressor gene whose loss contributes to lymphomagenesis by blocking plasma cell differentiation. |
