PMID- 10350329 OWN - NLM STAT- MEDLINE DCOM- 19990715 LR - 20190116 IS - 1042-8194 (Print) IS - 1026-8022 (Linking) VI - 34 IP - 1-2 DP - 1999 Jun TI - Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32). PG - 25-33 AB - B-cell non-Hodgkin's lymphoma (NHL) consists of heterogeneous subtypes based on histologic, immunophenotypic, and clinical findings. Recent advances in molecular biology have provided us new insights into the pathogenesis of this neoplasm at the genetic level, such as the deregulation of the protooncogenes adjoining the immunoglobulin gene (Ig) loci, which is a specific event in mature B-cell tumors. Moreover, involvement of certain protooncogenes corresponds to certain subtypes of NHL. Recently, we found that t(9;14)(p13;q32) chromosomal translocation associated with lymphoplasmacytic lymphoma (LPL) juxtaposes PAX-5 gene encoding for an essential transcription factor (BSAP: B-cell specific activator protein) for B-cell proliferation and differentiation to the Ig heavy chain gene (IgH) locus. This results in deregulated expression of the PAX-5 mRNA. We also developed a diagnostic FISH (fluorescence in situ hybridization) procedure which is able to detect 80% of the widely scattering 9p13 breakpoints involved in this translocation. Thus, an understanding of the PAX-5 gene's physiological role in B-cell development and the pathological role in tumorigenesis may lead to the optimal clinical treatment strategy for LPL and LPL-derived diffuse large cell lymphoma (DLCL). FAU - Iida, S AU - Iida S AD - Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY, USA. iida@med.nagoya-cu.ac.jp FAU - Rao, P H AU - Rao PH FAU - Ueda, R AU - Ueda R FAU - Chaganti, R S AU - Chaganti RS FAU - Dalla-Favera, R AU - Dalla-Favera R LA - eng PT - Journal Article PT - Review PL - United States TA - Leuk Lymphoma JT - Leukemia & lymphoma JID - 9007422 RN - 0 (DNA-Binding Proteins) RN - 0 (Nuclear Proteins) RN - 0 (PAX5 Transcription Factor) RN - 0 (PAX5 protein, human) RN - 0 (Transcription Factors) SB - IM MH - Animals MH - *Chromosomes, Human, Pair 14 MH - *Chromosomes, Human, Pair 9 MH - DNA-Binding Proteins/*genetics MH - Gene Rearrangement MH - Humans MH - Leukemia, Lymphocytic, Chronic, B-Cell/*genetics MH - Lymphoma, B-Cell/*genetics MH - Nuclear Proteins/*genetics MH - PAX5 Transcription Factor MH - *Transcription Factors MH - *Translocation, Genetic RF - 51 EDAT- 1999/06/01 00:00 MHDA- 1999/06/01 00:01 CRDT- 1999/06/01 00:00 PHST- 1999/06/01 00:00 [pubmed] PHST- 1999/06/01 00:01 [medline] PHST- 1999/06/01 00:00 [entrez] AID - 10.3109/10428199909083377 [doi] PST - ppublish SO - Leuk Lymphoma. 1999 Jun;34(1-2):25-33. doi: 10.3109/10428199909083377.