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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Detalhes bibliográficos
Publicado no:Biochim Biophys Acta
Main Authors: Besio, Roberta, Iula, Giusy, Garibaldi, Nadia, Cipolla, Lina, Sabbioneda, Simone, Biggiogera, Marco, Marini, Joan C., Rossi, Antonio, Forlino, Antonella
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier Pub. Co 2018
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5908783/
https://ncbi.nlm.nih.gov/pubmed/29432813
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbadis.2018.02.002
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