BCL6 (B-Cell Lymphoma 6)

3q27.3

706 amino acids; composed of a NH2-term BTB/POZ domain (amino acids 1-130 (32-99 according to Swiss-Prot) which mediates homodimerization and protein-protein interactions with other corepressors (including HDAC1 and NCOR2/SMRT to constitute a large repressing complex, another transcription repression domain (191-386), PEST sequences (300-417) with a KKYK motif (375-379), and six zinc finger at the C-term (518-541, 546-568, 574-596, 602-624, 630-652, 658-681), responsible for sequence specific DNA binding. Transcription repressor; recognizes the consensus sequence: TTCCT(A/C)GAA (Albagli-Curiel, 2003). Role in germinal centers of lymphoid follicles. BCL6 prevents ATM and TP53 to induce apoptosis in response to DNA rearrangements such as somatic hypermutation and class switch recombination. Therefore essential for normal B cell development.

LRMP (lymphoid restricted membrane protein)

12p12.1

LRMP is an integral membrane protein of the endoplasmic reticulum (ER), oriented on the ER membrane facing the cytosol. It is a lymphoid-restricted gene that is expressed in a developmentally regulated fashion (Behrens et al., 1994). It may be involved in diabetes susceptibility (Grimm et al., 2003). A V141L polymorphism of LRMP has been found associated with a higher mortality in patients with lung cancer (Manenti et al., 2006). LRMP has recently been found involved in a translocation t(8;12)(q24;p12) with a LRMP/MYC fusion gene in non Hodgkin lymphoma (Singh et al., 2012).