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A molecular basis for neurofibroma-associated skeletal manifestations in NF1

PURPOSE: Plexiform neurofibromas (NF) develop in children with Neurofibromatosis Type 1 (NF1) and can be associated with several skeletal co-morbidities. Preclinical mouse studies revealed Nf1 deficiency in osteoprogenitor cells disrupts, in a MEK-dependent manner, pyrophosphate (PPi) homeostasis an...

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Detalles Bibliográficos
Autores principales: Ma, Yun, Gross, Andrea, Dombi, Eva, Pemov, Alex, Choi, Kwangmin, Chaney, Katherine, Rhodes, Steven D., Angus, Steven P, Sciaky, Noah, Clapp, D Wade, Ratner, Nancy, Widemann, Brigitte C, Rios, Jonathan J, Elefteriou, Florent
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8106869/
https://www.ncbi.nlm.nih.gov/pubmed/32601387
http://dx.doi.org/10.1038/s41436-020-0885-3