Active receptor tyrosine kinases, but not Brachyury, are sufficient to trigger chordoma in zebrafish.

ABSTRACT: The aberrant activation of developmental processes triggers diverse cancer types. Chordoma is a rare, aggressive tumor arising from transformed notochord remnants. Several potentially oncogenic factors have been found to be deregulated in chordoma, yet causation remains uncertain. In particular, sustained expression of TBXT - encoding the notochord regulator protein brachyury - is hypothesized as a key driver of chordoma, yet experimental evidence is absent. Here, we employ a zebrafish chordoma model to identify the notochord-transforming potential of implicated genes in vivo We find that Brachyury, including a form with augmented transcriptional activity, is insufficient to initiate notochord hyperplasia. In contrast, the chordoma-implicated receptor tyrosine kinases (RTKs)

SUBMITTER: D'Agati G 

PROVIDER: S-EPMC6679381 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature