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Temporal order of RNase IIIb and loss-of-function mutations during development determines phenotype in pleuropulmonary blastoma / DICER1 syndrome: a unique variant of the two-hit tumor suppression model

Pleuropulmonary blastoma (PPB) is the most frequent pediatric lung tumor and often the first indication of a pleiotropic cancer predisposition,  DICER1 syndrome, comprising a range of other individually rare, benign and malignant tumors of childhood and early adulthood. The genetics of  DICER1-assoc...

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Detalles Bibliográficos
Autores principales: Brenneman, Mark, Field, Amanda, Yang, Jiandong, Williams, Gretchen, Doros, Leslie, Rossi, Christopher, Schultz, Kris Ann, Rosenberg, Avi, Ivanovich, Jennifer, Turner, Joyce, Gordish-Dressman, Heather, Stewart, Douglas, Yu, Weiying, Harris, Anne, Schoettler, Peter, Goodfellow, Paul, Dehner, Louis, Messinger, Yoav, Hill, D. Ashley
Formato: Online Artículo Texto
Lenguaje:English
Publicado: F1000 Research Limited 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4712775/
https://www.ncbi.nlm.nih.gov/pubmed/26925222
http://dx.doi.org/10.12688/f1000research.6746.2