Baculovial IAP repeat containing 3 (BIRC3) is a gene that encodes encodes the protein cellular inhibitor of apoptosis protein 2 (cIAP2), which inhibits apoptosis by binding to tumor necrosis factor receptor-associated factors TRAF1 and TRAF2; cIAP2 is also a negative regulator of NF-κB signaling in B-cells through its ubiquitin-ligase activity, which targets the downstream protein kinase MAP3K14 for degradation by the proteasome (Bertrand et al. 2011; Buggins et al. 2010; Conze, Zhao, and Ashwell 2010; Foà et al. 2013; Li, Yang, and Ashwell 2002; Rossi et al. 2012; Zarnegar et al. 2008; Zhou et al. 2013).
BIRC3 is localized to chromosome 11q22, whose deletion is associated with high risk disease in patients with chronic lymphocytic leukemia. BIRC3 mutations are observed in <5% of patients with CLL (Baliakas et al. 2015; Chiaretti et al. 2014; Cortese et al. 2014; Rossi et al. 2012; Xia et al. 2015), ~11% of patients with Splenic marginal zone lymphoma (Rossi et al. 2011) and 6-10% of mantle cell lymphoma cases (Rahal et al. 2014).