Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

Osteogenesis imperfecta (OI) types VII, VIII and IX, caused by recessive mutations in cartilage-associated protein (CRTAP), prolyl-3-hydroxylase 1 (P3H1) and cyclophilin B (PPIB), respectively, are characterized by the synthesis of overmodified collagen. The genes encode for the components of the en...

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Detalles Bibliográficos
Autores principales: Besio, Roberta, Garibaldi, Nadia, Leoni, Laura, Cipolla, Lina, Sabbioneda, Simone, Biggiogera, Marco, Mottes, Monica, Aglan, Mona, Otaify, Ghada A., Temtamy, Samia A., Rossi, Antonio, Forlino, Antonella
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2019
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6602311/
https://www.ncbi.nlm.nih.gov/pubmed/31171565
http://dx.doi.org/10.1242/dmm.038521

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6602311/
https://www.ncbi.nlm.nih.gov/pubmed/31171565
http://dx.doi.org/10.1242/dmm.038521

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