Deficiency in Perlecan/HSPG2 During Bone Development Enhances Osteogenesis and Decreases Quality of Adult Bone in Mice

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dc.citation.firstpage29en_US
dc.citation.issueNumber1en_US
dc.citation.journalTitleCalcified Tissue Internationalen_US
dc.citation.lastpage38en_US
dc.citation.volumeNumber95en_US
dc.contributor.authorLowe, Dylan A.en_US
dc.contributor.authorLepori-Bui, Nadiaen_US
dc.contributor.authorFomin, Peter V.en_US
dc.contributor.authorSloofman, Laura G.en_US
dc.contributor.authorZhou, Xiaozhouen_US
dc.contributor.authorFarach-Carson, Mary C.en_US
dc.contributor.authorWang, Liyunen_US
dc.contributor.authorKirn-Safran, Catherine B.en_US
dc.date.accessioned2017-08-17T19:19:41Zen_US
dc.date.available2017-08-17T19:19:41Zen_US
dc.date.issued2014en_US
dc.description.abstractPerlecan/HSPG2 (Pln) is a large heparan sulfate proteoglycan abundant in the extracellular matrix of cartilage and the lacunocanalicular space of adult bones. Although Pln function during cartilage development is critical, evidenced by deficiency disorders including Schwartz–Jampel Syndrome and dyssegmental dysplasia Silverman-Handmaker type, little is known about its function in development of bone shape and quality. The purpose of this study was to understand the contribution of Pln to bone geometric and mechanical properties. We used hypomorph mutant mice that secrete negligible amount of Pln into skeletal tissues and analyzed their adult bone properties using micro-computed tomography and three-point-bending tests. Bone shortening and widening in Pln mutants was observed and could be attributed to loss of growth plate organization and accelerated osteogenesis that was reflected by elevated cortical thickness at older ages. This effect was more pronounced in Pln mutant females, indicating a sex-specific effect of Pln deficiency on bone geometry. Additionally, mutant females, and to a lesser extent mutant males, increased their elastic modulus and bone mineral densities to counteract changes in bone shape, but at the expense of increased brittleness. In summary, Pln deficiency alters cartilage matrix patterning and, as we now show, coordinately influences bone formation and calcification.en_US
dc.identifier.citationLowe, Dylan A., Lepori-Bui, Nadia, Fomin, Peter V., et al.. "Deficiency in Perlecan/HSPG2 During Bone Development Enhances Osteogenesis and Decreases Quality of Adult Bone in Mice." <i>Calcified Tissue International,</i> 95, no. 1 (2014) Springer: 29-38. https://doi.org/10.1007/s00223-014-9859-2.en_US
dc.identifier.digitalBone_Developmenten_US
dc.identifier.doihttps://doi.org/10.1007/s00223-014-9859-2en_US
dc.identifier.urihttps://hdl.handle.net/1911/97333en_US
dc.language.isoengen_US
dc.publisherSpringeren_US
dc.rightsThis is an author's peer-reviewed final manuscript, as accepted by the publisher. The published article is copyrighted by Springer.en_US
dc.subject.keywordPerlecanen_US
dc.subject.keywordHeparan Sulfateen_US
dc.subject.keywordProteoglycanen_US
dc.subject.keywordBone Qualityen_US
dc.subject.keywordSchwartz-Jampel Syndromeen_US
dc.subject.keyworddyssegmental dysplasia Silverman-Handmakeren_US
dc.titleDeficiency in Perlecan/HSPG2 During Bone Development Enhances Osteogenesis and Decreases Quality of Adult Bone in Miceen_US
dc.typeJournal articleen_US
dc.type.dcmiTexten_US
dc.type.publicationpost-printen_US
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