A BAFF-R mutation associated with non-Hodgkin lymphoma alters TRAF recruitment and reveals new insights into BAFF-R signaling.

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Hildebrand, JM; Luo, Z; Manske, MK; Price-Troska, T; Ziesmer, SC; Lin, W; Hostager, BS; Slager, SL; Witzig, TE; Ansell, SM; ...Date
2010-11-22Source Title
Journal of Experimental MedicinePublisher
Rockefeller University PressUniversity of Melbourne Author/s
Hildebrand, JoanneAffiliation
Medical Biology (W.E.H.I.)Metadata
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Hildebrand, J. M., Luo, Z., Manske, M. K., Price-Troska, T., Ziesmer, S. C., Lin, W., Hostager, B. S., Slager, S. L., Witzig, T. E., Ansell, S. M., Cerhan, J. R., Bishop, G. A. & Novak, A. J. (2010). A BAFF-R mutation associated with non-Hodgkin lymphoma alters TRAF recruitment and reveals new insights into BAFF-R signaling.. J Exp Med, 207 (12), pp.2569-2579. https://doi.org/10.1084/jem.20100857.Access Status
Open AccessOpen Access at PMC
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989778Abstract
The cytokine B cell activating factor (BAFF) and its receptor, BAFF receptor (BAFF-R), modulate signaling cascades critical for B cell development and survival. We identified a novel mutation in TNFRSF13C, the gene encoding human BAFF-R, that is present in both tumor and germline tissue from a subset of patients with non-Hodgkin lymphoma. This mutation encodes a His159Tyr substitution in the cytoplasmic tail of BAFF-R adjacent to the TRAF3 binding motif. Signaling through this mutant BAFF-R results in increased NF-κB1 and NF-κB2 activity and increased immunoglobulin production compared with the wild-type (WT) BAFF-R. This correlates with increased TRAF2, TRAF3, and TRAF6 recruitment to His159Tyr BAFF-R. In addition, we document a requirement for TRAF6 in WT BAFF-R signaling. Together, these data identify a novel lymphoma-associated mutation in human BAFF-R that results in NF-κB activation and reveals TRAF6 as a necessary component of normal BAFF-R signaling.
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