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Mutations in the FK506 Binding Protein 10 (<jats:italic>FKBP10<\/jats:italic>), gene encoding the 65\u2010kDa protein FKBP65, cause a recessive form of OI and Bruck syndrome, the latter being characterized by joint contractures in addition to low bone mass. We previously showed that <jats:italic>Fkbp10<\/jats:italic> expression is limited to bone, tendon, and ligaments in postnatal tissues. Furthermore, in both patients and <jats:italic>Fkbp10<\/jats:italic> knockout mice, collagen telopeptide hydroxylysine crosslinking is dramatically reduced. To further characterize the bone specific contributions of <jats:italic>Fkbp10<\/jats:italic>, we conditionally ablated FKBP65 in <jats:italic>Fkbp10<jats:sup>fl\/fl<\/jats:sup><\/jats:italic> mice (<jats:italic>Mus musculus<\/jats:italic>; C57BL\/6) using the osteoblast\u2010specific <jats:italic>Col1a1<\/jats:italic> 2.3\u2010kb Cre recombinase. Using \u03bcCT, histomorphometry and quantitative backscattered electron imaging, we found minimal alterations in the quantity of bone and no differences in the degree of bone matrix mineralization in this model. However, mass spectroscopy (MS) of bone collagen demonstrated a decrease in mature, hydroxylysine\u2010aldehyde crosslinking. Furthermore, bone of mutant mice exhibits a reduction in mineral\u2010to\u2010matrix ratio and in crystal size as shown by Raman spectroscopy and small\u2010angle X\u2010ray scattering, respectively. Importantly, abnormalities in bone quality were associated with impaired bone biomechanical strength in mutant femurs compared with those of wild\u2010type littermates. Taken together, these data suggest that the altered collagen crosslinking through <jats:italic>Fkbp10<\/jats:italic> ablation in osteoblasts primarily leads to a qualitative defect in the skeleton. \u00a9 2017 American Society for Bone and Mineral Research.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jbmr.3108","type":"journal-article","created":{"date-parts":[[2017,2,16]],"date-time":"2017-02-16T11:52:52Z","timestamp":1487245972000},"page":"1354-1367","source":"Crossref","is-referenced-by-count":25,"title":["<i>Fkbp10<\/i> Deletion in Osteoblasts Leads to Qualitative Defects in Bone"],"prefix":"10.1093","volume":"32","author":[{"given":"Caressa D","family":"Lietman","sequence":"first","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joohyun","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingo","family":"Grafe","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuqing","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hao","family":"Ding","sequence":"additional","affiliation":[{"name":"Department of Nanomedicine and Biomedical Engineering University of Texas Health Science Center at Houston Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaohong","family":"Bi","sequence":"additional","affiliation":[{"name":"Department of Nanomedicine and Biomedical Engineering University of Texas Health Science Center at Houston Houston TX 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":"Nadja","family":"Fratzl\u2010Zelman","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute of Osteology Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling 1st Med. Dept. Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Roschger","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute of Osteology Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling 1st Med. Dept. Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Klaushofer","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute of Osteology Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling 1st Med. Dept. Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Wagermaier","sequence":"additional","affiliation":[{"name":"Department of Biomaterials Max Planck Institute of Colloids and Interfaces Research Campus Golm Potsdam Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingo","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Biomaterials Max Planck Institute of Colloids and Interfaces Research Campus Golm Potsdam Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Fratzl","sequence":"additional","affiliation":[{"name":"Department of Biomaterials Max Planck Institute of Colloids and Interfaces Research Campus Golm Potsdam Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jyoti","family":"Rai","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics and Sports Medicine University of Washington Seattle WA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MaryAnn","family":"Weis","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics and Sports Medicine University of Washington Seattle WA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Eyre","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics and Sports Medicine University of Washington Seattle WA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas R","family":"Keene","sequence":"additional","affiliation":[{"name":"Micro\u2010Imaging Center Shriners Hospital for Children Portland OR USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah","family":"Krakow","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery David Geffen School of Medicine at UCLA Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brendan H","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2017,3,20]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00223-015-9985-5"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00223-013-9723-9"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.2356"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/8756-3282(95)00328-B"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/112.4.708"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00198-009-1066-z"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00198-012-2228-y"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00198-005-2035-9"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)16051-0"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20799"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.250"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2006.08.039"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2010.02.022"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00441-009-0872-0"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000117"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi400760f"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1139\/o10-137"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M802535200"},{"issue":"11","key":"e_1_2_7_20_1","first-page":"3925","article-title":"Developmental regulation of FKBP65","volume":"11","author":"Patterson CE","year":"2000","journal-title":"An ER\u2010localized extracellular matrix binding\u2010protein. 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