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PLoS One ; 8(6): e65677, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-23776525

RESUMEN

Non-syndromic cleft lip/palate (NSCL/P) is a complex, frequent congenital malformation, determined by the interplay between genetic and environmental factors during embryonic development. Previous findings have appointed an aetiological overlap between NSCL/P and cancer, and alterations in similar biological pathways may underpin both conditions. Here, using a combination of transcriptomic profiling and functional approaches, we report that NSCL/P dental pulp stem cells exhibit dysregulation of a co-expressed gene network mainly associated with DNA double-strand break repair and cell cycle control (p = 2.88×10(-2)-5.02×10(-9)). This network included important genes for these cellular processes, such as BRCA1, RAD51, and MSH2, which are predicted to be regulated by transcription factor E2F1. Functional assays support these findings, revealing that NSCL/P cells accumulate DNA double-strand breaks upon exposure to H2O2. Furthermore, we show that E2f1, Brca1 and Rad51 are co-expressed in the developing embryonic orofacial primordia, and may act as a molecular hub playing a role in lip and palate morphogenesis. In conclusion, we show for the first time that cellular defences against DNA damage may take part in determining the susceptibility to NSCL/P. These results are in accordance with the hypothesis of aetiological overlap between this malformation and cancer, and suggest a new pathogenic mechanism for the disease.


Asunto(s)
Labio Leporino/genética , Fisura del Paladar/genética , Roturas del ADN de Doble Cadena , Pulpa Dental/citología , Redes Reguladoras de Genes/genética , Células Madre/metabolismo , Proteína BRCA1/genética , Ciclo Celular/genética , Células Cultivadas , Niño , Cartilla de ADN/genética , Reparación del ADN/genética , Factor de Transcripción E2F1/genética , Factor de Transcripción E2F1/metabolismo , Citometría de Flujo , Perfilación de la Expresión Génica , Humanos , Hibridación in Situ , Proteína 2 Homóloga a MutS/genética , Análisis por Matrices de Proteínas , Recombinasa Rad51/genética , Reacción en Cadena en Tiempo Real de la Polimerasa
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