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

• Main Authors: Roberta Besio (Author), Nadia Garibaldi (Author), Laura Leoni (Author), Lina Cipolla (Author), Simone Sabbioneda (Author), Marco Biggiogera (Author), Monica Mottes (Author), Mona Aglan (Author), Ghada A. Otaify (Author), Samia A. Temtamy (Author), Antonio Rossi (Author), Antonella Forlino (Author)
• Format: Book
• Published: The Company of Biologists, 2019-06-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...