4-PBA ameliorates cellular homeostasis in fibroblasts from osteogenesis imperfecta patients by enhancing autophagy and stimulating protein secretion

The clinical phenotype in osteogenesis imperfecta (OI) is attributed to the dominant negative function of mutant type I collagen molecules in the extracellular matrix, by altering its structure and function. Intracellular retention of mutant collagen has also been reported, but its effect on cellula...

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Detalles Bibliográficos
Autores principales: Besio, Roberta, Iula, Giusy, Garibaldi, Nadia, Cipolla, Lina, Sabbioneda, Simone, Biggiogera, Marco, Marini, Joan C., Rossi, Antonio, Forlino, Antonella
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Pub. Co 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5908783/
https://www.ncbi.nlm.nih.gov/pubmed/29432813
http://dx.doi.org/10.1016/j.bbadis.2018.02.002

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