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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Bibliographic Details

Published in:	Disease Models & Mechanisms
Main Authors:	Roberta Besio, Nadia Garibaldi, Laura Leoni, Lina Cipolla, Simone Sabbioneda, Marco Biggiogera, Monica Mottes, Mona Aglan, Ghada A. Otaify, Samia A. Temtamy, Antonio Rossi, Antonella Forlino
Format:	Article
Language:	English
Published:	The Company of Biologists 2019-06-01
Subjects:	Osteogenesis imperfecta Endoplasmic reticulum stress Chemical chaperone Unfolded protein response 4-PBA
Online Access:	http://dmm.biologists.org/content/12/6/dmm038521