BCL6 Breakapart LPH035 - Detail

Rearrangements of the BCL6 gene have been observed in 20-40% of diffuse large B cell lymphoma (DLCL) and in 5-15% of follicular lymphoma (FL)^1,2.

This translocation results in the deregulation of BCL6 and may involve the IGH, IGK and IGL genes, although other non-Ig partner genes have also been identified ^2,3. BCL6 encodes a 96 kD nuclear phosphoprotein belonging to the pox virus zinc finger (POZ)/zinc finger (ZF) family of transcription factors⁴. BCL6 functions as a transcriptional repressor and plays an important regulatory role in lymphoid cell development and function. In the B-cell lineage the BCL6 protein is expressed only in germinal centre B cells⁵. Translocations involving band 3q27 affect a 4 kb major breakpoint region (MBR) of BCL6 in the first non-coding exon and 5' region of the first intron which are common in diffuse large B Cell Lymphomas. Recently an alternative breakpoint cluster region (ABR) located between 245 and 285kb 5' of BCL6 was identified in Non-Hodgkins Lymphomas (NHL) which may be preferentially associated with Follicular Lymphoma⁶.

Reference:/Bibliographie/Literatur/Bibliografia

1. Ohno H et al (1997) Leuk Lymphoma 27: 53-54
2. Ueda C et al (2002) Leuk Lymphoma 43: 1375-81
3. Akasaka H et al (2000) Cancer Res 60: 2335-41
4. Chang CC et al (1996) PNAS 93: 6947-52
5. Cattoretti G et al (1995) Blood 85:45-53
6. Bosga-Bouwer AG et al (2005) Genes Chrom Cancer 44: 301-304