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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 emerging as a contributor to OI pathogenesis. Herein, we used 4-phenylbutiric acid (4-PBA), an established chemical chaperone, to determine if treatment of Aga2+\/\u2212 mice, a model for moderately severe OI due to a Col1a1 structural mutation, could attenuate the phenotype. In vitro, Aga2+\/\u2212 osteoblasts show increased protein kinase RNA-like endoplasmic reticulum kinase (PERK) activation protein levels, which improved upon treatment with 4-PBA. The in vivo data demonstrate that a postweaning 5-week 4-PBA treatment increased total body length and weight, decreased fracture incidence, increased femoral bone volume fraction (BV\/TV), and increased cortical thickness. These findings were associated with in vivo evidence of decreased bone-derived protein levels of the ER stress markers binding immunoglobulin protein (BiP), CCAAT\/\u2212enhancer-binding protein homologous protein (CHOP), and activating transcription factor 4 (ATF4) as well as increased levels of the autophagosome marker light chain 3A\/B (LC3A\/B). Genetic ablation of CHOP in Aga2+\/\u2212 mice resulted in increased severity of the Aga2+\/\u2212 phenotype, suggesting that the reduction in CHOP observed in vitro after treatment is a consequence rather than a cause of reduced ER stress. These findings suggest the potential use of chemical chaperones as an adjunct treatment for forms of OI associated with ER stress. \u00a9 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).<\/jats:p>","DOI":"10.1002\/jbmr.4501","type":"journal-article","created":{"date-parts":[[2022,1,8]],"date-time":"2022-01-08T08:53:44Z","timestamp":1641632024000},"page":"675-686","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["4-PBA Treatment Improves Bone Phenotypes in the Aga2 Mouse Model of Osteogenesis Imperfecta"],"prefix":"10.1093","volume":"37","author":[{"given":"Ivan","family":"Duran","sequence":"first","affiliation":[{"name":"Networking Biomedical Research Center in Bioengineering Biomaterials and Nanomedicine (CIBER-BBN), Andalusian Centre for Nanomedicine and Biotechnology (BIONAND) M\u00e1laga Spain"},{"name":"Laboratory of Bioengineering and Tissue Regeneration (LABRET), Department of Cell Biology, Genetics and Physiology University of M\u00e1laga, Institute of Biomedical Research in Malaga (IBIMA) M\u00e1laga Spain"},{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Zieba","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiana","family":"Csukasi","sequence":"additional","affiliation":[{"name":"Networking Biomedical Research Center in Bioengineering Biomaterials and Nanomedicine (CIBER-BBN), Andalusian Centre for Nanomedicine and Biotechnology (BIONAND) M\u00e1laga Spain"},{"name":"Laboratory of Bioengineering and Tissue Regeneration (LABRET), Department of Cell Biology, Genetics and Physiology University of M\u00e1laga, Institute of Biomedical Research in Malaga (IBIMA) M\u00e1laga Spain"},{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge H.","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davis","family":"Wachtell","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya","family":"Barad","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian","family":"Dawson","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bohumil","family":"Fafilek","sequence":"additional","affiliation":[{"name":"International Clinical Research Center St. Anne's University Hospital Brno Czech Republic"},{"name":"Department of Biology, Faculty of Medicine Masaryk University Brno Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina M.","family":"Jacobsen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Harvard Medical School Boston MA USA"},{"name":"Divisions of Endocrinology and Genetics and Genomics Boston Children's Hospital Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine G.","family":"Ambrose","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery University of Texas Health Science Center at Houston Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel H.","family":"Cohn","sequence":"additional","affiliation":[{"name":"Department of Molecular Cell and Developmental Biology University of California at Los Angeles Los Angeles CA USA"},{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at University of California at Los Angeles Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavel","family":"Krejci","sequence":"additional","affiliation":[{"name":"International Clinical Research Center St. Anne's University Hospital Brno Czech Republic"},{"name":"Department of Biology, Faculty of Medicine Masaryk University Brno Czech 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