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Expression of an active Gα(s) mutant in skeletal stem cells is sufficient and necessary for fibrous dysplasia initiation and maintenance

Fibrous dysplasia (FD) is a disease caused by postzygotic activating mutations of GNAS (R201C and R201H) that encode the α-subunit of the G(s) stimulatory protein. FD is characterized by the development of areas of abnormal fibroosseous tissue in the bones, resulting in skeletal deformities, fractur...

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Detalles Bibliográficos
Autores principales: Zhao, Xuefeng, Deng, Peng, Iglesias-Bartolome, Ramiro, Amornphimoltham, Panomwat, Steffen, Dana J., Jin, Yunyun, Molinolo, Alfredo A., de Castro, Luis Fernandez, Ovejero, Diana, Yuan, Quan, Chen, Qianming, Han, Xianglong, Bai, Ding, Taylor, Susan S., Yang, Yingzi, Collins, Michael T., Gutkind, J. Silvio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: National Academy of Sciences 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5776975/
https://www.ncbi.nlm.nih.gov/pubmed/29282319
http://dx.doi.org/10.1073/pnas.1713710115