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Thomas M Braxton School of Biosciences, Cardiff University, Cardiff, UK

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Dionne E A Sarpong School of Biosciences, Cardiff University, Cardiff, UK

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Janine L Dovey School of Biosciences, Cardiff University, Cardiff, UK

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Anne Guillou IGF, CNRS, INSERM, University of Montpellier, Montpellier, France

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Bronwen A J Evans School of Medicine, Cardiff University, Cardiff, UK

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Juan M Castellano Physiology Section, Faculty of Medicine, University of Cordoba, and Instituto Maimonides de Investigacion Biomedica de Cordoba (IMBIC), Cordoba, Spain

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Bethany E Keenan School of Engineering, Cardiff University, Cardiff, UK

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Saja Baraghithy Obesity and Metabolism Laboratory, Institute for Drug Research, School of Pharmacy, Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem, Israel

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Sam L Evans School of Engineering, Cardiff University, Cardiff, UK

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Manuel Tena-Sempere Physiology Section, Faculty of Medicine, University of Cordoba, and Instituto Maimonides de Investigacion Biomedica de Cordoba (IMBIC), Cordoba, Spain
CIBER Fisiopatologia de la Obesidad y Nutrición (CIBEROBN), Instituto de Salud Carlos III, Cordoba, Spain

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Patrice Mollard IGF, CNRS, INSERM, University of Montpellier, Montpellier, France

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Joseph Tam Obesity and Metabolism Laboratory, Institute for Drug Research, School of Pharmacy, Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem, Israel

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Timothy Wells School of Biosciences, Cardiff University, Cardiff, UK

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Human Prader–Willi syndrome (PWS) is characterised by impairments of multiple systems including the growth hormone (GH) axis and skeletal growth. To address our lack of knowledge of the influence of PWS on skeletal integrity in mice, we have characterised the endocrine and skeletal phenotype of the PWS-IC del mouse model for ‘full’ PWS and determined the impact of thermoneutrality. Tibial length, epiphyseal plate width and marrow adiposity were reduced by 6, 18 and 79% in male PWS-IC del mice, with osteoclast density being unaffected. Similar reductions in femoral length accompanied a 32% reduction in mid-diaphyseal cortical diameter. Distal femoral Tb.N was reduced by 62%, with individual trabeculae being less plate-like and the lattice being more fragmented (Tb.Pf increased by 63%). Cortical strength (ultimate moment) was reduced by 26% as a result of reductions in calcified tissue strength and the geometric contribution. GH and prolactin contents in PWS-IC del pituitaries were reduced in proportion to their smaller pituitary size, with circulating IGF-1 concentration reduced by 37–47%. Conversely, while pituitary luteinising hormone content was halved, circulating gonadotropin concentrations were unaffected. Although longitudinal growth, marrow adiposity and femoral geometry were unaffected by thermoneutrality, strengthened calcified tissue reversed the weakened cortex of PWS-IC del femora. While underactivity of the GH axis may be due to loss of Snord116 expression and impaired limb bone geometry and strength due to loss of Magel2 expression, comprehensive analysis of skeletal integrity in the single gene deletion models is required. Our data imply that thermoneutrality may ameliorate the elevated fracture risk associated with PWS.

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