{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T01:09:29Z","timestamp":1790644169551,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2020,4,17]],"date-time":"2020-04-17T00:00:00Z","timestamp":1587081600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Intramural Research Program of the NIH, National Cancer Institute, Center for Cancer Research","award":["ZIA BC011028"],"award-info":[{"award-number":["ZIA BC011028"]}]},{"name":"Intramural Research Program of the NIH, National Cancer Institute, Center for Cancer Research","award":["ZIA BC011038"],"award-info":[{"award-number":["ZIA BC011038"]}]},{"name":"Intramural Research Program of the NIH, National Cancer Institute, Center for Cancer Research","award":["ZIA BC011089"],"award-info":[{"award-number":["ZIA BC011089"]}]},{"name":"Intramural Research Program of the NIH, National Cancer Institute, Center for Cancer Research","award":["ZIC BC011044"],"award-info":[{"award-number":["ZIC BC011044"]}]},{"name":"NCATS Division of Preclinical Innovation"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp; Cancer"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Renal medullary carcinoma (RMC) is a rare, aggressive disease that predominantly afflicts individuals of African or Mediterranean descent with sickle cell trait. RMC comprises 1% of all renal cell carcinoma diagnoses with a median overall survival of 13\u2009months. Patients are typically young (median age\u201422) and male (male:female ratio of 2:1) and tumors are characterized by complete loss of expression of the SMARCB1 tumor suppressor protein. Due to the low incidence of RMC and the disease's aggressiveness, treatment decisions are often based on case reports. Thus, it is critical to develop preclinical models of RMC to better understand the pathogenesis of this disease and to identify effective forms of therapy. Two novel cell line models, UOK353 and UOK360, were derived from primary RMCs that both demonstrated the characteristic SMARCB1 loss. Both cell lines overexpressed <jats:italic>EZH2<\/jats:italic> and other members of the polycomb repressive complex and EZH2 inhibition in RMC tumor spheroids resulted in decreased viability. High throughput drug screening of both cell lines revealed several additional candidate compounds, including bortezomib that had both in vitro and in vivo antitumor activity. The activity of bortezomib was shown to be partially dependent on increased oxidative stress as addition of the N\u2010acetyl cysteine antioxidant reduced the effect on cell proliferation. Combining bortezomib and cisplatin further decreased cell viability both in vitro and in vivo that single agent bortezomib treatment. The UOK353 and UOK360 cell lines represent novel preclinical models for the development of effective forms of therapy for RMC patients.<\/jats:p>","DOI":"10.1002\/gcc.22847","type":"journal-article","created":{"date-parts":[[2020,4,7]],"date-time":"2020-04-07T16:07:00Z","timestamp":1586275620000},"page":"472-483","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Novel renal medullary carcinoma cell lines, <scp>UOK353<\/scp> and <scp>UOK360<\/scp>, provide preclinical tools to identify new therapeutic treatments"],"prefix":"10.1002","volume":"59","author":[{"given":"Darmood","family":"Wei","sequence":"first","affiliation":[{"name":"Urologic Oncology Branch, Center for Cancer Research National Cancer Institute, National Institutes of Health  Bethesda Maryland United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youfeng","family":"Yang","sequence":"additional","affiliation":[{"name":"Urologic 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