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Article

4-PBA Treatment Improves Bone Phenotypes in the Aga2 Mouse Model of Osteogenesis Imperfecta.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Apr 2022

Duran Ivan, Zieba Jennifer, Csukasi Fabiana, Martin Jorge H, Wachtell Davis, Barad Maya, Dawson Brian, Fafilek Bohumil, Jacobsen Christina M, Ambrose Catherine G, Cohn Daniel H, Krejci Pavel, Lee Brendan H, Krakow Deborah

Abstract excerpt

Osteogenesis imperfecta (OI) is a genetically heterogenous disorder most often due to heterozygosity for mutations in the type I procollagen genes, COL1A1 or COL1A2. The disorder is characterized by bone fragility leading to increased fracture incidence and long-bone deformities. Although multiple mechanisms underlie OI, endoplasmic reticulum (ER) stress as a cellular response to defective collagen trafficking is...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 34997935
DOI: 10.1002/jbmr.4501
PMCID: PMC9018561

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