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As GATA\u20103 is transcriptionally regulated by NF\u2010\u03baB, we sought to determine the extent to which proteasomal inhibition impairs NF\u2010\u03baB activation and GATA\u20103 expression and cell viability in malignant T cells. Proteasome inhibition, NF\u2010\u03baB activity, GATA\u20103 expression, and cell viability were examined in patient\u2010derived cell lines and primary T\u2010cell lymphoma specimens ex vivo treated with the oral proteasome inhibitor ixazomib. Significant reductions in cell viability, NF\u2010\u03baB activation, and GATA\u20103 expression were observed preclinically in ixazomib\u2010treated cells. Therefore, an investigator\u2010initiated, single\u2010center, phase II study with this agent in patients with relapsed\/refractory CTCL\/PTCL was conducted. Concordant with our preclinical observations, a significant reduction in NF\u2010\u03baB activation and GATA\u20103 expression was observed in an exceptional responder following one month of treatment with ixazomib. While ixazomib had limited activity in this small and heterogeneous cohort of patients, inhibition of the NF\u2010\u03baB\/GATA\u20103 axis in a single exceptional responder suggests that ixazomib may have utility in appropriately selected patients or in combination with other agents.<\/jats:p>","DOI":"10.1002\/ajh.24895","type":"journal-article","created":{"date-parts":[[2017,8,26]],"date-time":"2017-08-26T08:32:36Z","timestamp":1503736356000},"page":"1287-1294","source":"Crossref","is-referenced-by-count":29,"title":["A single center phase II study of ixazomib in patients with relapsed or refractory cutaneous or peripheral T\u2010cell lymphomas"],"prefix":"10.1002","volume":"92","author":[{"given":"Philip S.","family":"Boonstra","sequence":"first","affiliation":[{"name":"Department of Biostatistics University of Michigan Ann Arbor Michigan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Avery","family":"Polk","sequence":"additional","affiliation":[{"name":"Division 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