Regular ArticleLoss- and gain-of-function mutations reveal an important role of BSAP (Pax-5) at the start and end of B cell differentiation☆
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Long-Term Survival of Primary Intracranial Plasmablastic Lymphoma: Case Report and Review of the Literature
2017, World NeurosurgeryCitation Excerpt :PBLs express epithelial membrane antigen-2 and lack the germinal center-associated antigen BCL-6.18,23,24 PAX-5 (B-cell differentiation antigen) is usually negative, which expresses from the precursor B-cell to mature B cells but disappears in terminal differentiation to plasma cells.25,26 These results provide evidence that PBL might be a plasmacytic differentiation.
GEMMs addressing Pax5 loss-of-function in childhood pB-ALL
2016, European Journal of Medical GeneticsB cell linker protein (BLNK) is a selective target of repression by PAX5-PML protein in the differentiation block that leads to the development of acute lymphoblastic leukemia
2016, Journal of Biological ChemistryCitation Excerpt :PAX5 is expressed exclusively from the pro-B to mature B cell stage and is down-regulated during terminal differentiation into plasma cells (1). PAX5 is indispensable for B lineage commitment by the transcriptional activation of B lineage-specific genes (2), such as CD19 (3), CD79A (4), and B cell linker protein (BLNK) (5), and its target disruption has been shown to cause B lymphoid maturation arrest at the pro-B cell stage (6). Previous studies have identified the PAX5 gene as the most frequent target of somatic mutations in childhood and adult B-progenitor acute lymphoblastic leukemia (ALL), being altered in 38.9% and 34% of cases, respectively (7, 8), and these findings further emphasized the essential role of PAX5 in the proper development of B cells.
Expression of essential B cell development genes in horses with common variable immunodeficiency
2012, Molecular ImmunologyAberrant somatic hypermutation in tumor cells of nodular-lymphocyte- predominant and classic Hodgkin lymphoma
2006, BloodCitation Excerpt :This finding is also consistent with the hypothesis that a qualitative deficit of the SHM machinery (eg, loss of target specificity) rather than an overall increase in its activity may be responsible for these mutations in B-cell lymphomas. Activation of c-MYC, RhoH/TTF, PIM1, and PAX5 may be relevant for B-cell lymphomagenesis14 because these genes encode for signal transducers (PIM1 and RhoH/TTF) and transcription factors (PAX5 and c-MYC) involved in B-cell development/differentiation or in the regulation of proliferation and apoptosis, and they have also been implicated in lymphoma-associated chromosomal translocations.30-33 In NLPHL and cHL, aberrant SHM may alter the function of these genes by at least 2 modalities.
Loss of Pax5 promotes plasma cell differentiation
2006, Immunity
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