{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T18:40:42Z","timestamp":1790793642660,"version":"4.1.0"},"reference-count":43,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2019,9,30]],"date-time":"2019-09-30T00:00:00Z","timestamp":1569801600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Hereditary cancers with cancer-predisposing mutations represent unique models of human oncogenesis, as a driving oncogenic event is present in germline. Currently, there are no satisfactory models to study these malignancies. We report the generation of IPSC from the somatic cells of a patient with hereditary c-met-mutated papillary renal cell carcinoma (PRCC). From these cells we have generated spontaneous aggregates organizing in structures which expressed kidney markers such as PODXL and Six2. These structures expressed PRCC markers both in vitro and in vivo in NSG mice. Gene-expression profiling showed striking molecular similarities with signatures found in a large cohort of PRCC tumor samples. This analysis, applied to primary cancers with and without c-met mutation, showed overexpression of the BHLHE40 and KDM4C only in the c-met-mutated PRCC tumors, as predicted by c-met-mutated embryoid bodies transcriptome. These data therefore represent the first proof of concept of \u201chereditary renal cancer in a dish\u201d model using c-met-mutated iPSC-derived embryoid bodies, opening new perspectives for discovery of novel predictive progression markers and for drug-screening for future precision-medicine strategies.<\/jats:p>","DOI":"10.3390\/ijms20194867","type":"journal-article","created":{"date-parts":[[2019,9,30]],"date-time":"2019-09-30T13:16:41Z","timestamp":1569849401000},"page":"4867","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["iPSC-Derived Embryoid Bodies as Models of c-Met-Mutated Hereditary Papillary Renal Cell Carcinoma"],"prefix":"10.3390","volume":"20","author":[{"given":"Jin Wook","family":"Hwang","sequence":"first","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7679-2524","authenticated-orcid":false,"given":"Christophe","family":"Desterke","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"F\u00e9raud","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9859-9773","authenticated-orcid":false,"given":"Stephane","family":"Richard","sequence":"additional","affiliation":[{"name":"R\u00e9seau National de R\u00e9f\u00e9rence pour Cancers Rares de l\u2019Adulte PREDIR, labellis\u00e9 par l\u2019INCa, et Service d\u2019Urologie, AP-HP, H\u00f4pital Bic\u00eatre, 94270 Le Kremlin-Bic\u00eatre, France; G\u00e9n\u00e9tique Oncologique EPHE, PSL Universit\u00e9, INSERM UMR 1186, Gustave Roussy, Facult\u00e9 de M\u00e9decine et Universit\u00e9 Paris-Sud, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Ferlicot","sequence":"additional","affiliation":[{"name":"INSERM, UMR 1186, Gustave Roussy, Paris-Sud University, Paris-Saclay University, 94800 Villejuif, France"},{"name":"Department of Pathology, Bic\u00eatre Hospital, AP-HP, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginie","family":"Verkarre","sequence":"additional","affiliation":[{"name":"Service d\u2019Anatomie Pathologique, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, 75015 Paris, France"},{"name":"Facult\u00e9 de m\u00e9decine, Universit\u00e9 Paris Descartes, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Jacques","family":"Patard","sequence":"additional","affiliation":[{"name":"Service d\u2019Urologie, Centre Hospitalier de Mont de Marsan, 40024 Mont de Marsan, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Loisel-Duwattez","sequence":"additional","affiliation":[{"name":"INSERM U1195, Universit\u00e9 Paris Sud, Facult\u00e9 de M\u00e9decine, APHP, Service de Neurologie, H\u00f4pital Bic\u00eatre, 94276 le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5002-5490","authenticated-orcid":false,"given":"Adlen","family":"Foudi","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"},{"name":"ATIP Avenir INSERM UMR-S 935, Universit\u00e9 Paris Sud, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Griscelli","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"},{"name":"INGESTEM National IPSC Infrastructure, 94800 Villejuif, France"},{"name":"Paris Descartes University, Faculty Sorbonne Paris Cit\u00e9, Facult\u00e9 des Sciences Pharmaceutiques et Biologiques, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3557-4499","authenticated-orcid":false,"given":"Annelise","family":"Bennaceur-Griscelli","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"},{"name":"INGESTEM National IPSC Infrastructure, 94800 Villejuif, France"},{"name":"Division of Hematology, Paris Sud University Hospitals, Le Kremlin Bic\u00eatre 75006, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4861-0137","authenticated-orcid":false,"given":"Ali G","family":"Turhan","sequence":"additional","affiliation":[{"name":"INSERM UMR-S 935 and ESTeam Paris Sud, Universit\u00e9 Paris Sud, 94800 Villejuif, France"},{"name":"INGESTEM National IPSC Infrastructure, 94800 Villejuif, France"},{"name":"Division of Hematology, Paris Sud University Hospitals, Le Kremlin Bic\u00eatre 75006, 94800 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,9,30]]},"reference":[{"key":"ref_1","first-page":"260847","article-title":"Identification, Evaluation, and Treatment of Patients with Hereditary Cancer Risk within the United States","volume":"2013","author":"Cragun","year":"2013","journal-title":"ISRN 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