Microstructural heterogenity in human bone affected by X-linked hypophosphatemia
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https://doi.esrf.fr/10.15151/ESRF-ES-2242566855
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资源简介：
The genetic disease X-linked hypophosphatemia (XLH) causes skeletal deformities and impaired bone mineralization. This is believed to be related to increased levels of fibroblast growth factor 23 and local accumulation of mineralization-inhibiting osteopontin. The latter leads to hypomineralized lesions in bone and a highly heterogeneous mineralization pattern. The impact of XLH on the bone hydroxyapatite (HAP) biomineral properties and the collagen matrix has not been studied in human bone that has different remodelling schemes than rodents and present a more diverse set of mutations than the most studied mouse model. We hypothesize that the microstructural manifestations of XLH on bone mineralization are coupled with changes in the HAP crystallite properties as well as changes in the organization of the collagen matrix. Therefore, we aim to quantify the mineral and collagen bone matrix of XLH bone using 2D scanning SAXS/WAXS/XRF on a large number of human iliac crest bone samples.
提供机构：
Aarhus University , Department of Chemistry, Langelandsgade 140, Aarhus C, 8000 Aarhus, Denmark; Aarhus University, Interdisciplinary Nanoscience Center, Gustav Wieds Vej 14, Aarhus C, 8000 Aarhus, Denmark; Aarhus University , Department of Chemistry, Langelandsgade 140 Aarhus C, 8000, Aarhus, DENMARK
创建时间：
2028-01-01
