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J Pathol. 2012 Nov;228(3):261-5. doi: 10.1002/path.4102.

'The chicken or the egg?' dilemma strikes back for the controlling mechanism in chordoma(#).

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Department of Molecular Cell Biology, Leiden University Medical Center, Einthovenweg 20, 2330 RC, Leiden, The Netherlands.


Chordoma is a rare malignant tumour of bone showing notochordal differentiation with characteristic expression of the transcription factor brachyury (T). Next to giving insight into its differentiation spectrum, the expression of T can be used as an adjunct diagnostic tool. The expression of brachyury in chordoma is necessary to maintain cell proliferation in chordoma cell lines, indicating that in chordoma it might be a master regulator of oncogenesis. Identification and mapping of the full gene regulatory network in a recent work in The Journal of Pathology by Nelson and colleagues not only shed light on involved pathways but also indicated pathways for targeted therapy, including brachyury itself.

[Indexed for MEDLINE]

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