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Molecular and cellular characterizations of human cherubism: disease aggressiveness depends on osteoclast differentiation

BACKGROUND: Cherubism is a rare autosomal dominant disorder of the jaws caused by mutation of the SH3BP2 gene. The bone is replaced by a fibrous granuloma containing multinucleated giant cells. Cells of the cherubism granuloma have never been systematically analyzed. Hence, the aim of this study was...

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Detalles Bibliográficos
Autores principales: Kadlub, Natacha, Sessiecq, Quentin, Mandavit, Marion, L’Hermine, Aurore Coulomb, Badoual, Cecile, Galmiche, Louise, Berdal, Ariane, Descroix, Vianney, Picard, Arnaud, Coudert, Amélie E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6148781/
https://www.ncbi.nlm.nih.gov/pubmed/30236129
http://dx.doi.org/10.1186/s13023-018-0907-2