{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T02:50:55Z","timestamp":1772160655415,"version":"3.50.1"},"reference-count":41,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2022,6,14]],"date-time":"2022-06-14T00:00:00Z","timestamp":1655164800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["GNT 1049863, GNT 1154819"],"award-info":[{"award-number":["GNT 1049863, GNT 1154819"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cellular Molecular Medi"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The inherited brittle bone disease osteogenesis imperfecta (OI) is commonly caused by\n                    <jats:italic>COL1A1<\/jats:italic>\n                    and\n                    <jats:italic>COL1A2<\/jats:italic>\n                    mutations that disrupt the collagen I triple helix. This causes intracellular endoplasmic reticulum (ER) retention of the misfolded collagen and can result in a pathological ER stress response. A therapeutic approach to reduce this toxic mutant load could be to stimulate mutant collagen degradation by manipulating autophagy and\/or ER\u2010associated degradation. Since carbamazepine (CBZ) both stimulates autophagy of misfolded collagen X and improves skeletal pathology in a metaphyseal chondrodysplasia model, we tested the effect of CBZ on bone structure and strength in 3\u2010week\u2010old male OI\n                    <jats:italic>Col1a2<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>+\/p.G610C<\/jats:italic>\n                    <\/jats:sup>\n                    and control mice. Treatment for 3 or 6\u2009weeks with CBZ, at the dose effective in metaphyseal chondrodysplasia, provided no therapeutic benefit to\n                    <jats:italic>Col1a2<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>+\/p.G610C<\/jats:italic>\n                    <\/jats:sup>\n                    mouse bone structure, strength or composition, measured by micro\u2010computed tomography, three point bending tests and Fourier\u2010transform infrared microspectroscopy. In control mice, however, CBZ treatment for 6\u2009weeks impaired femur growth and led to lower femoral cortical and trabecular bone mass. These data, showing the negative impact of CBZ treatment on the developing mouse bones, raise important issues which must be considered in any human clinical applications of CBZ in growing individuals.\n                  <\/jats:p>","DOI":"10.1111\/jcmm.17437","type":"journal-article","created":{"date-parts":[[2022,6,14]],"date-time":"2022-06-14T22:15:13Z","timestamp":1655244913000},"page":"4021-4031","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The effect of carbamazepine on bone structure and strength in control and osteogenesis imperfecta (\n                    <i>\n                      Col1a2\n                      <sup>+\/p.G610C<\/sup>\n                    <\/i>\n                    ) mice"],"prefix":"10.1111","volume":"26","author":[{"given":"Martha","family":"Blank","sequence":"first","affiliation":[{"name":"Bone Cell Biology and Disease Unit St. Vincent's Institute of Medical Research Melbourne Victoria Australia"},{"name":"Department of Medicine The University of Melbourne, St. Vincent's Hospital Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Narelle E.","family":"McGregor","sequence":"additional","affiliation":[{"name":"Bone Cell Biology and Disease Unit St. Vincent's Institute of Medical Research Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lynn","family":"Rowley","sequence":"additional","affiliation":[{"name":"Musculoskeletal Research Murdoch Children's Research Institute Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louise H. 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