Targeting oxidative stress in embryonal rhabdomyosarcoma.
Targeting oxidative stress in embryonal rhabdomyosarcoma.
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DOI：
10.1016/j.ccr.2013.11.002
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发表时间：
2013-12-09
期刊：
影响因子：
50.3
通讯作者：
St. Jude Children’s Research Hospital–Washington University Pediatric Cancer Genome Project
中科院分区：
文献类型：
--
作者：
Chen X;Stewart E;Shelat AA;Qu C;Bahrami A;Hatley M;Wu G;Bradley C;McEvoy J;Pappo A;Spunt S;Valentine MB;Valentine V;Krafcik F;Lang WH;Wierdl M;Tsurkan L;Tolleman V;Federico SM;Morton C;Lu C;Ding L;Easton J;Rusch M;Nagahawatte P;Wang J;Parker M;Wei L;Hedlund E;Finkelstein D;Edmonson M;Shurtleff S;Boggs K;Mulder H;Yergeau D;Skapek S;Hawkins DS;Ramirez N;Potter PM;Sandoval JA;Davidoff AM;Mardis ER;Wilson RK;Zhang J;Downing JR;Dyer MA;St. Jude Children’s Research Hospital–Washington University Pediatric Cancer Genome Project
横纹肌肉瘤是一种软组织肉瘤，具有发育中的骨骼肌的分子和细胞特征。横纹肌肉瘤有胚胎型和肺泡型两种主要的组织学亚型，每种亚型都有不同的临床、分子和遗传学特征。基因组分析表明，胚胎型肿瘤比肺泡型肿瘤具有更多的结构和拷贝数变异。RAS/NF1通路的突变与中、高危胚胎性横纹肌肉瘤(ERMS)显著相关。相比之下，肺泡型横纹肌肉瘤(ARMS)总体上遗传损伤较少，且没有已知的反复突变的癌症共识基因。为了确定ERMS的治疗方法，我们开发了原位异种肿瘤移植，并在我们的研究中对其进行了测序。对来自这些异种移植物的原代培养物进行高通量筛选，确定氧化应激是ERMS的一种治疗途径。
Rhabdomyosarcoma is a soft-tissue sarcoma with molecular and cellular features of developing skeletal muscle. Rhabdomyosarcoma has two major histological subtypes, embryonal and alveolar, each with distinct clinical, molecular, and genetic features. Genomic analysis show that embryonal tumors have more structural and copy number variations than alveolar tumors. Mutations in the RAS/NF1 pathway are significantly associated with intermediate- and high-risk embryonal rhabdomyosarcomas (ERMS). In contrast, alveolar rhabdomyosarcoma (ARMS) have fewer genetic lesions overall and no known recurrently mutated cancer consensus genes. To identify therapeutics for ERMS, we developed and characterized orthotopic xenografts of tumors that were sequenced in our study. High throughput screening of primary cultures derived from those xenografts identified oxidative stress as a pathway of therapeutic relevance for ERMS.
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影响因子：
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作者：
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通讯作者：
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影响因子：
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DOI：
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发表时间：
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影响因子：
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作者：
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影响因子：
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作者：
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