From notochord formation to hereditary chordoma: the many roles of Brachyury.
From notochord formation to hereditary chordoma: the many roles of Brachyury.
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DOI：
10.1155/2013/826435
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发表时间：
2013
影响因子：
--
通讯作者：
Di Gregorio A
中科院分区：
文献类型：
--
作者：
Nibu Y;José-Edwards DS;Di Gregorio A
脊索瘤是一种罕见的，但往往是恶性的，骨癌，优先影响轴骨和颅底。这些肿瘤是散发性和遗传性的，似乎更频繁地发生后的第四个十年的生活;然而，现代技术已经增加了儿童脊索瘤的检测。脊索瘤起源于脊索的残余物，脊索是在脊柱之前的主要胚胎轴结构，并且与脊索细胞共享组织学特征和特征基因的表达。一个这样的基因是Brachyury，其编码序列特异性转录因子。几十年来，Brachyury被认为是脊索形成的主要调节因子，最近作为脊索炎的生物标志物和病原体，并因此作为有希望的治疗靶点，Brachyury引起了人们的兴趣。在这里，我们回顾了脉络膜的主要特点，分子标记物，以及目前可用于早期检测和可能的治疗这种癌症的临床方法。特别是，我们报告目前的知识的作用Brachyury和其可能的作用机制，在脊索形成和chorophysis病因。
Chordoma is a rare, but often malignant, bone cancer that preferentially affects the axial skeleton and the skull base. These tumors are both sporadic and hereditary and appear to occur more frequently after the fourth decade of life; however, modern technologies have increased the detection of pediatric chordomas. Chordomas originate from remnants of the notochord, the main embryonic axial structure that precedes the backbone, and share with notochord cells both histological features and the expression of characteristic genes. One such gene is Brachyury, which encodes for a sequence-specific transcription factor. Known for decades as a main regulator of notochord formation, Brachyury has recently gained interest as a biomarker and causative agent of chordoma, and therefore as a promising therapeutic target. Here, we review the main characteristics of chordoma, the molecular markers, and the clinical approaches currently available for the early detection and possible treatment of this cancer. In particular, we report on the current knowledge of the role of Brachyury and of its possible mechanisms of action in both notochord formation and chordoma etiogenesis.
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影响因子：
3.7
作者：
Evans AL;Faial T;Gilchrist MJ;Down T;Vallier L;Pedersen RA;Wardle FC;Smith JC
通讯作者：
Smith JC
影响因子：
5.3
作者：
Correa, RG;Tergaonkar, V;Verma, IM
通讯作者：
Verma, IM
影响因子：
45.3
作者：
Chugh, R;Dunn, R;Baker, LH
通讯作者：
Baker, LH
影响因子：
--
作者：
Chesley, P
通讯作者：
Chesley, P
影响因子：
3
作者：
Boriani, S;Bandiera, S;Weinstein, JN
通讯作者：
Weinstein, JN