Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10845741 | Seminars in Cancer Biology | 2005 | 9 Pages |
Abstract
The ETV6-NTRK3 (TEL-TRKC) gene fusion was discovered by breakpoint analysis of the t(12;15)(p13;q25) translocation associated with congenital fibrosarcoma, a pediatric soft tissue malignancy. ETV6-NTRK3 (EN) encodes the sterile alpha motif oligomerization domain of the ETV6 (TEL) transcription factor linked to the protein tyrosine kinase domain of the neurotrophin-3 receptor NTRK3 (TRKC). The EN chimeric oncoprotein links to multiple signaling cascades including Ras-MAP kinase and PI3K-AKT through the IRS-1 adapter protein. Recent evidence indicates that a functional insulin-like growth factor 1 receptor axis and higher order polymer formation are essential for EN oncogenesis. EN has been detected in other malignancies, including secretory breast carcinoma. This chimeric oncoprotein is therefore unique in being expressed in tumors derived from multiple cell lineages.
Keywords
PI3KERKPTKNTRK3Mitogen-activated protein kinase/extracellular signal-regulated kinase kinaseETV6TELIRS-1PTBMAPKTranslocationinsulin receptor substrate-1phosphotyrosine binding domainPhosphatidylinositol 3-kinaseMEKProtein tyrosine kinasemitogen-activated protein kinaseextracellular signal-regulated kinaseinsulin-like growth factor-I receptor
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Authors
Chris L. Lannon, Poul H.B. Sorensen,