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The WNT1^(G177C) mutation specifically affects skeletal integrity in a mouse model of osteogenesis imperfecta type XV

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Bone Research 2021年第4期9卷 593-605页

作者： Nele Vollersen Wenbo Zhao Tim Rolvien Fabiola Lange Felix Nikolai Schmidt Stephan Sonntag Doron Shmerling Simon von Kroge Kilian Elia Stockhausen Ahmed Sharaf Michaela Schweizer Meliha Karsak Bjorn Busse ernesto bockamp Oliver Semler Michael Amling Ralf Oheim Thorsten Schinke Timur Alexander Yorgan Department of Osteology and Biomechanics University Medical Center Hamburg-Eppendorf20246 HamburgGermany PolyGene AG 8153 RumlangSwitzerland ETH Phenomics Center(EPIC) ETH Zurich8092 ZurichSwitzerland Neuronal and Cellular Signal Transduction Center for Molecular Neurobiology Hamburg(ZMNH)University Medical Center Hamburg-Eppendorf20246 HamburgGermany Center for Molecular Neurobiology Hamburg(ZMNH) University Medical CenterHamburg-Eppendorf20246 HamburgGermany Institute for Translational Immunology and Research Center for Immunotherapy University Medical CenterJohannes Gutenberg UniversityD 55131 MainzGermany Faculty of Medicine and University Hospital Cologne Department of PediatricsUniversity of Cologne50937 CologneGermany

The recent identification of homozygous WNT1 mutations in individuals with osteogenesis imperfecta type XV(OI-XV)has suggested that WNT1 is a key ligand promoting the differentiation and function of bone-forming *** such aninfluence was supported by subsequent studies,a mouse model of OI-XV remained to be ***,we introduced a previously identified disease-causing mutation(G177C)into the murine Wnt1 *** Wnt1^(G177C/G177C)mice were viable and did not display defects in brain development,but the majority of 24-week-old Wnt1^(G177C/G177C)mice had skeletal *** increased bone fragility was not fully explained by reduced bone mass but also by impaired bone matrix ***,the homozygous presence of the G177C mutation did not interfere with the osteoanabolic influence of either parathyroid hormone injection or activating mutation of LRP5,the latter mimicking the effect of sclerostin ***,transcriptomic analyses revealed that short-term administration of WNT1 to osteogenic cells induced not only the expression of canonical WNT signaling targets but also the expression of genes encoding extracellular matrix *** together,our data demonstrate that regulating bone matrix quality is a primary function of *** further suggest that individuals with WNT1 mutations should profit from existing osteoanabolic therapies.

关键词： WNT1 skeletal impaired

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