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◇ bioRxiv2026-09-17· cancer biology

Oncogenic fusions induce an extensive cancer-restricted cryptic proteome in Ewing sarcoma

L. A. Broeils, J. Pilet, M. K. Koshkina, J. Li, S. Grossetete, E. G. A. Westerink, O. Jay, L. Smit, T. Luo, E. Narmanli, A. P. Pinheiro Lopes, F. H. Geyer, S. A. G. Engels, C. Henon, K. Aljakouch, J. Krijgsveld, I. Jamail, S. Baulande, S. Lameiras, M. J. Carreno-Gonzalez, G. W. Omlor, J. M. Mudge, V. U. Nguyen, M. I. Swirski, H. Tjeldnes, E. Valen, B. Lehner, U. Dirksen, W. Faigle, A. I. Lalanne, O. Lantz, K. Laud-Duval, C. Michail, M. VanInsberghe, A. van Oudenaarden, N. Hahnen, N. Gmelin, J. H. M. Merks, F. Alkan, J. J. Waterfall, T. G. P. Gruenewald

原始摘要(英文原文)· Original abstract
How tumors generate a cryptic "dark" proteome absent from healthy tissues, and whether it can be reversibly activated, remains unclear. In Ewing sarcoma, all tumors are driven by EWSR1::ETS fusions, making it a tractable model to study dark proteome activation. Integrating matched transcriptomes, translatomes and proteomes from 48 patient tumors with long-read RNA sequencing, single-cell Ribo-seq, proteomics and immunopeptidomics in cell line models, we show that EWSR1::FLI1 acts as a reversible switch recurrently inducing hundreds of cancer-specific neoproteins. Many arise from canonical coding regions via intragenic transcription start sites, generating truncated or out-of-frame neoproteins. We uncover a fusion-dependent increase in ribosomal readthrough into poly(A) tails, generating a stable 80-amino-acid TRPM4 neoprotein that, despite lacking a stop codon, is the most abundant tumor-specific microprotein across patients. Proteomic, immunopeptidomic, and immunofluorescence analyses validate neoproteins as tumor-restricted antigens, revealing a therapeutically actionable cancer dark proteome controlled by one oncogenic fusion.
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Oncogenic fusions induce an extensive cancer-restricted cryptic proteome in Ewing sarcoma — 科研速览 Science Skim