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Unsuspected osteochondroma-like outgrowths in the cranial base of Hereditary Multiple Exostoses patients and modeling and treatment with a BMP antagonist in mice

Hereditary Multiple Exostoses (HME) is a rare pediatric disorder caused by loss-of-function mutations in the genes encoding the heparan sulfate (HS)-synthesizing enzymes EXT1 or EXT2. HME is characterized by formation of cartilaginous outgrowths—called osteochondromas- next to the growth plates of m...

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Detalles Bibliográficos
Autores principales: Sinha, Sayantani, Mundy, Christina, Bechtold, Till, Sgariglia, Federica, Ibrahim, Mazen M., Billings, Paul C., Carroll, Kristen, Koyama, Eiki, Jones, Kevin B., Pacifici, Maurizio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5425227/
https://www.ncbi.nlm.nih.gov/pubmed/28445472
http://dx.doi.org/10.1371/journal.pgen.1006742