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Biomed Res Int. 2013;2013:826435. doi: 10.1155/2013/826435. Epub 2013 Mar 31.

From notochord formation to hereditary chordoma: the many roles of Brachyury.

Author information

1
Department of Cell and Developmental Biology, Weill Medical College of Cornell University, New York, NY 10065, USA. yun2001@med.cornell.edu

Abstract

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.

PMID:
23662285
PMCID:
PMC3626178
DOI:
10.1155/2013/826435
[Indexed for MEDLINE]
Free PMC Article

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