{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T12:51:14Z","timestamp":1790254274951,"version":"4.1.0"},"reference-count":65,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2013,7,31]],"date-time":"2013-07-31T00:00:00Z","timestamp":1375228800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,1,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>\u2003<\/jats:title>\n                    <jats:p>The bone formation inhibitor sclerostin encoded by SOST binds in vitro to low-density lipoprotein receptor-related protein (LRP) 5\/6 Wnt co-receptors, thereby inhibiting Wnt\/\u03b2-catenin signaling, a central pathway of skeletal homeostasis. Lrp5\/LRP5 deficiency results in osteoporosis-pseudoglioma (OPPG), whereas Sost\/SOST deficiency induces lifelong bone gain in mice and humans. Here, we analyzed the bone phenotype of mice lacking Sost (Sost\u2212\/\u2212), Lrp5 (Lrp5\u2212\/\u2212), or both (Sost\u2212\/\u2212;Lrp5\u2212\/\u2212) to elucidate the mechanism of action of Sost in vivo. Sost deficiency\u2013induced bone gain was significantly blunted in Sost\u2212\/\u2212;Lrp5\u2212\/\u2212 mice. Yet the Lrp5 OPPG phenotype was fully rescued in Sost\u2212\/\u2212;Lrp5\u2212\/\u2212 mice and most bone parameters were elevated relative to wild-type. To test whether the remaining bone increases in Sost\u2212\/\u2212;Lrp5\u2212\/\u2212 animals depend on Lrp6, we treated wild-type, Sost\u2212\/\u2212, and Sost\u2212\/\u2212;Lrp5\u2212\/\u2212 mice with distinct Lrp6 function blocking antibodies. Selective blockage of Wnt1 class\u2013mediated Lrp6 signaling reduced cancellous bone mass and density in wild-type mice. Surprisingly, it reversed the abnormal bone gain in Sost\u2212\/\u2212 and Sost\u2212\/\u2212;Lrp5\u2212\/\u2212 mice to wild-type levels irrespective of enhancement or blockage of Wnt3a class-mediated Lrp6 activity. Thus, whereas Sost deficiency\u2013induced bone anabolism partially requires Lrp5, it fully depends on Wnt1 class\u2013induced Lrp6 activity. These findings indicate: first, that OPPG syndrome patients suffering from LRP5 loss-of-function should benefit from principles antagonizing SOST\/sclerostin action; and second, that therapeutic WNT signaling inhibitors may stop the debilitating bone overgrowth in sclerosing disorders related to SOST deficiency, such as sclerosteosis, van Buchem disease, and autosomal dominant craniodiaphyseal dysplasia, which are rare disorders without viable treatment options. \u00a9 2014 American Society for Bone and Mineral Research.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jbmr.2059","type":"journal-article","created":{"date-parts":[[2013,7,31]],"date-time":"2013-07-31T04:11:08Z","timestamp":1375243868000},"page":"29-42","source":"Crossref","is-referenced-by-count":69,"title":["Reversing\n                    <i>LRP<\/i>\n                    <i>5<\/i>\n                    -Dependent Osteoporosis and\n                    <i>SOST<\/i>\n                    Deficiency\u2013Induced Sclerosing Bone Disorders by Altering WNT Signaling Activity"],"prefix":"10.1093","volume":"29","author":[{"given":"Ming-Kang","family":"Chang","sequence":"first","affiliation":[{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ina","family":"Kramer","sequence":"additional","affiliation":[{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hansjoerg","family":"Keller","sequence":"additional","affiliation":[{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan H","family":"Gooi","sequence":"additional","affiliation":[{"name":"University of Melbourne, Department of Zoology, Reproduction & DevelopmentParkville3010VictoriaAustralia"},{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Collett","sequence":"additional","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (National Institute of Health and Medical Research) U942Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), H\u00f4pital Lariboisi\u00e8re, Service de BiochimieParisFrance"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Jenkins","sequence":"additional","affiliation":[{"name":"OncologyNovartis Institutes for BioMedical ResearchCambridgeMAUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seth A","family":"Ettenberg","sequence":"additional","affiliation":[{"name":"OncologyNovartis Institutes for BioMedical ResearchCambridgeMAUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng","family":"Cong","sequence":"additional","affiliation":[{"name":"Developmental and Molecular PathwaysNovartis Institutes for BioMedical ResearchCambridgeMAUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Halleux","sequence":"additional","affiliation":[{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Kneissel","sequence":"additional","affiliation":[{"name":"Musculoskeletal Disease AreaNovartis Institutes for BioMedical ResearchBaselSwitzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2013,7,31]]},"reference":[{"key":"2024020318592548000_jbmr2059-bib-0001","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1016\/j.devcel.2005.02.017","article-title":"Canonical Wnt signaling in differentiated osteoblasts controls osteoclast differentiation","volume":"8","author":"Glass","year":"2005","journal-title":"Dev Cell."},{"key":"2024020318592548000_jbmr2059-bib-0002","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1016\/j.devcel.2005.02.013","article-title":"Canonical Wnt\/beta-catenin signaling prevents osteoblasts from differentiating into chondrocytes","volume":"8","author":"Hill","year":"2005","journal-title":"Dev Cell."},{"key":"2024020318592548000_jbmr2059-bib-0003","doi-asserted-by":"crossref","first-page":"21162","DOI":"10.1074\/jbc.M501900200","article-title":"Essential role of beta-catenin in postnatal bone acquisition","volume":"280","author":"Holmen","year":"2005","journal-title":"J Biol Chem."},{"key":"2024020318592548000_jbmr2059-bib-0004","doi-asserted-by":"crossref","first-page":"3071","DOI":"10.1128\/MCB.01428-09","article-title":"Osteocyte Wnt\/beta-catenin signaling is required for normal bone homeostasis","volume":"30","author":"Kramer","year":"2010","journal-title":"Mol Cell Biol."},{"key":"2024020318592548000_jbmr2059-bib-0005","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/S0092-8674(01)00571-2","article-title":"LDL receptor-related protein 5 (LRP5) affects bone accrual and eye development","volume":"107","author":"Gong","year":"2001","journal-title":"Cell."},{"key":"2024020318592548000_jbmr2059-bib-0006","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1359\/jbmr.040907","article-title":"Decreased BMD and limb deformities in mice carrying mutations in both Lrp5 and Lrp6","volume":"19","author":"Holmen","year":"2004","journal-title":"J Bone Miner 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factors","volume":"315","author":"Mani","year":"2007","journal-title":"Science."},{"key":"2024020318592548000_jbmr2059-bib-0010","doi-asserted-by":"crossref","first-page":"e63323","DOI":"10.1371\/journal.pone.0063323","article-title":"Lrp5 and Lrp6 exert overlapping functions in osteoblasts during postnatal bone acquisition","volume":"8","author":"Riddle","year":"2013","journal-title":"PLOS One."},{"key":"2024020318592548000_jbmr2059-bib-0011","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1359\/jbmr.2003.18.6.960","article-title":"High bone mass in mice expressing a mutant LRP5 gene","volume":"18","author":"Babij","year":"2003","journal-title":"J Bone Miner Res."},{"key":"2024020318592548000_jbmr2059-bib-0012","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1056\/NEJMoa013444","article-title":"High bone density due to a mutation in LDL-receptor-related protein 5","volume":"346","author":"Boyden","year":"2002","journal-title":"New Engl J Med."},{"key":"2024020318592548000_jbmr2059-bib-0013","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1086\/338450","article-title":"A mutation in the LDL receptor-related protein 5 gene results in the autosomal dominant high-bone-mass trait","volume":"70","author":"Little","year":"2002","journal-title":"Am J Hum Genet."},{"key":"2024020318592548000_jbmr2059-bib-0014","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1182\/blood-2008-03-145169","article-title":"The role of Dickkopf-1 in bone development, homeostasis, and disease","volume":"113","author":"Pinzone","year":"2009","journal-title":"Blood."},{"key":"2024020318592548000_jbmr2059-bib-0015","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1093\/hmg\/10.5.537","article-title":"Increased bone density in sclerosteosis is due to the deficiency of a novel secreted protein (SOST)","volume":"10","author":"Balemans","year":"2001","journal-title":"Hum Mol Genet."},{"key":"2024020318592548000_jbmr2059-bib-0016","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1086\/318811","article-title":"Bone dysplasia sclerosteosis results from loss of the SOST gene product, a novel cystine knot-containing protein","volume":"68","author":"Brunkow","year":"2001","journal-title":"Am J Hum Genet."},{"key":"2024020318592548000_jbmr2059-bib-0017","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1136\/jmg.39.2.91","article-title":"Identification of a 52 kb deletion downstream of the SOST gene in patients with van Buchem disease","volume":"39","author":"Balemans","year":"2002","journal-title":"J Med Genet."},{"key":"2024020318592548000_jbmr2059-bib-0018","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1101\/gr.3437105","article-title":"Genomic deletion of a long-range bone enhancer misregulates sclerostin in Van Buchem disease","volume":"15","author":"Loots","year":"2005","journal-title":"Genome 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