Topics: Single Cells
Origin: IP
Project type: Expertise
Name of Applicant: Vanessa RIBES
Date of application: 13-04-2026
Unit: Other
Location: Phone: 0649450882
@ Mail: vanessa.ribes@ijm.fr
Project context and summary: Our project aims to understand the early emergence steps of a pediatric cancer, named fusion-negative (FN) rhabdomyosarcoma (RMS). A key feature of FN-RMS is the frequent mutations in regulators of the RAS/MAPK pathways, including NF1, FGFR4, PIK3CA, and RAS family members, leading to abnormal activation of these pathways. However, whether their activity in embryonic lineages can lead to tumorigenesis is not well characterized. To investigate this, we use the chick neural tube as a model and test whether embryonic neurogenic populations, particularly neural crest cells, can be at the origin of FN-RMS. We have shown that Ras carrying constitutively active mutations can induce invasive neoplasia in chick neural tube that presents cell-to-cell variability in terms of identity. We aim to further investigate this cellular heterogeneity with the Pasteur platform.
Related team publications:BMP2 and BMP7 cooperate with H3.3K27M to promote quiescence and invasiveness in pediatric diffuse midline gliomas. Huchede P, Meyer S, Berthelot C, Hamadou M, Bertrand-Chapel A, Rakotomalala A, Manceau L, Tomine J, Lespinasse N, Lewandowski P, Cordier-Bussat M, Broutier L, Dutour A, Rochet I, Blay JY, Degletagne C, Attignon V, Montero-Carcaboso A, Le Grand M, Pasquier E, Vasiljevic A, Gilardi-Hebenstreit P, Meignan S, Leblond P, Ribes V, Cosset E, Castets M. Elife. 2024 Oct 7;12:RP91313. doi: 10.7554/eLife.91313. PMID: 39373720
Divergent transcriptional and transforming properties of PAX3-FOXO1 and PAX7-FOXO1 paralogs. Manceau L, Richard Albert J, Lollini PL, Greenberg MVC, Gilardi-Hebenstreit P, Ribes V. PLoS Genet. 2022 May 23;18(5):e1009782. doi: 10.1371/journal.pgen.1009782. eCollection 2022 May. PMID: 35604932
Gonzalez Curto G*, Der Vartanian A*, Frarma YE*, Manceau L*, Baldi L*, Prisco S, Elarouci N, Causeret F, Korenkov D, Rigolet M, Aurade F, De Reynies A, Contremoulins V, Relaix F, Faklaris O, Briscoe J, Gilardi-Hebenstreit P, Ribes V°. The PAX-FOXO1s trigger fast trans-differentiation of chick embryonic neural cells into alveolar rhabdomyosarcoma with tissue invasive properties limited by S phase entry inhibition. PLoS Genet. 2020. Nov 11;16(11):e1009164. doi: 10.1371/journal.pgen.1009164. PMID: 33175861. 15 citations.
Manager: marc.monot@pasteur.frStatus: Kick-off meeting
