{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:20:52Z","timestamp":1784557252354,"version":"3.55.0"},"reference-count":84,"publisher":"Bioscientifica","issue":"3","content-domain":{"domain":["joe.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:p>\n                    Osteogenesis imperfecta (OI) is the most common heritable bone fragility disorder, usually caused by dominant mutations in genes coding for collagen type I alpha chains,\n                    <jats:italic>COL1A1<\/jats:italic>\n                    or\n                    <jats:italic>COL1A2<\/jats:italic>\n                    . Osteocalcin (OCN) is now recognized as a bone-derived regulator of insulin secretion and sensitivity and glucose homeostasis. Since OI is associated with increased rates of bone formation and resorption, we hypothesized that the levels of undercarboxylated OCN are increased in OI. The objective of this study was to determine changes in OCN and to elucidate the metabolic phenotype in the Col1a1\n                    <jats:sup>Jrt\/+<\/jats:sup>\n                    mouse, a model of dominant OI caused by a\n                    <jats:italic>Col1a1<\/jats:italic>\n                    mutation. Circulating levels of undercarboxylated OCN were higher in 4-week-old OI mice and normal by 8 weeks of age. Young OI animals exhibited a sex-dependent metabolic phenotype, including increased insulin levels in males, improved glucose tolerance in females, lower levels of random glucose and low adiposity in both sexes. The rates of O\n                    <jats:sub>2<\/jats:sub>\n                    consumption and CO\n                    <jats:sub>2<\/jats:sub>\n                    production, as well as energy expenditure assessed using indirect calorimetry were significantly increased in OI animals of both sexes, whereas respiratory exchange ratio was significantly higher in OI males only. Although OI mice have significant physical impairment that may contribute to metabolic differences, we specifically accounted for movement and compared OI and WT animals during the periods of similar activity levels. Taken together, our data strongly suggest that OI animals have alterations in whole body energy metabolism that are consistent with the action of undercarboxylated osteocalcin.\n                  <\/jats:p>","DOI":"10.1530\/joe-17-0335","type":"journal-article","created":{"date-parts":[[2017,7,17]],"date-time":"2017-07-17T20:10:20Z","timestamp":1500322220000},"page":"279-289","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-5","source":"Crossref","is-referenced-by-count":31,"title":["Metabolic phenotype in the mouse model of osteogenesis imperfecta"],"prefix":"10.1530","volume":"234","author":[{"given":"Iris","family":"Boraschi-Diaz","sequence":"first","affiliation":[{"name":"1Shriners Hospital for Children-Canada, Montreal, Quebec, Canada"},{"name":"2Faculty of Dentistry, McGill University, Montreal, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josephine T","family":"Tauer","sequence":"additional","affiliation":[{"name":"1Shriners Hospital for Children-Canada, Montreal, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omar","family":"El-Rifai","sequence":"additional","affiliation":[{"name":"3Unit\u00e9 de Recherche en Physiologie Int\u00e9grative et Mol\u00e9culaire, Institut de Recherches Cliniques de Montr\u00e9al, Montr\u00e9al, Qu\u00e9bec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Guillemette","sequence":"additional","affiliation":[{"name":"1Shriners Hospital for Children-Canada, Montreal, Quebec, Canada"},{"name":"4D\u00e9partement de Math\u00e9matiques, Universit\u00e9 du Qu\u00e9bec \u00e0 Montr\u00e9al, Montr\u00e9al, Qu\u00e9bec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevi\u00e8ve","family":"Lefebvre","sequence":"additional","affiliation":[{"name":"4D\u00e9partement de Math\u00e9matiques, Universit\u00e9 du Qu\u00e9bec \u00e0 Montr\u00e9al, Montr\u00e9al, Qu\u00e9bec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Rauch","sequence":"additional","affiliation":[{"name":"1Shriners Hospital for Children-Canada, Montreal, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Ferron","sequence":"additional","affiliation":[{"name":"3Unit\u00e9 de Recherche en Physiologie Int\u00e9grative et Mol\u00e9culaire, Institut de Recherches Cliniques de Montr\u00e9al, Montr\u00e9al, Qu\u00e9bec, Canada"},{"name":"5D\u00e9partements de M\u00e9decine et de Biochimie et M\u00e9decine Mol\u00e9culaire, Universit\u00e9 de Montr\u00e9al, Montr\u00e9al, Qu\u00e9bec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svetlana V","family":"Komarova","sequence":"additional","affiliation":[{"name":"1Shriners Hospital for Children-Canada, Montreal, Quebec, Canada"},{"name":"2Faculty of Dentistry, McGill University, Montreal, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"ref211","doi-asserted-by":"crossref","first-page":"1594","DOI":"10.1210\/endo.143.5.8807","article-title":"Osteoblastic response to the defective matrix in the osteogenesis imperfecta murine (oim) mouse","volume":"143","year":"2002","journal-title":"Endocrinology"},{"key":"ref121","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.bbrc.2010.06.008","article-title":"An ELISA-based method to quantify osteocalcin carboxylation in mice","volume":"397","year":"2010b","journal-title":"Biochemical and Biophysical Research Communications"},{"key":"ref321","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1359\/jbmr.091109","article-title":"Relationship between genotype and skeletal phenotype in children and adolescents with osteogenesis imperfecta","volume":"25","year":"2010","journal-title":"Journal of Bone and Mineral Research"},{"key":"ref751","volume-title":"Methods of 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