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Naji, M. |
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Motta, Antonella |
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Aletan, Dirar |
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Mohamed, Tarek |
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Ertürk, Emre |
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Taccardi, Nicola |
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Kononenko, Denys |
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Petrov, R. H. | Madrid |
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Alshaaer, Mazen | Brussels |
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Bih, L. |
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Casati, R. |
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Muller, Hermance |
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Kočí, Jan | Prague |
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Šuljagić, Marija |
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Kalteremidou, Kalliopi-Artemi | Brussels |
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Azam, Siraj |
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Ospanova, Alyiya |
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Blanpain, Bart |
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Ali, M. A. |
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Popa, V. |
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Rančić, M. |
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Ollier, Nadège |
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Azevedo, Nuno Monteiro |
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Landes, Michael |
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Rignanese, Gian-Marco |
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Raum, Kay
in Cooperation with on an Cooperation-Score of 37%
Topics
Publications (14/14 displayed)
- 2024The respective and dependent effects of scattering and bone matrix absorption on ultrasound attenuation in cortical bone.citations
- 2021Anisotropic elastic properties of human cortical bone tissue inferred from inverse homogenization and resonant ultrasound spectroscopycitations
- 2020Cortical thinning and accumulation of large cortical pores in the tibia reflect local structural deterioration of the femoral neckcitations
- 2019Large cortical bone pores in the tibia are associated with proximal femur strengthcitations
- 2019Acoustic diffusion constant of cortical bone: Numerical simulation study of the effect of pore size and pore density on multiple scattering.citations
- 2016Multimodal correlative investigation of the interplaying micro-architecture, chemical composition and mechanical properties of human cortical bone tissue reveals predominant role of fibrillar organization in determining microelastic tissue properties.citations
- 2015Distribution of mesoscale elastic properties and mass density in the human femoral shaft.citations
- 2014Ultrasound to assess bone quality.citations
- 20143D Raman mapping of the collagen fibril orientation in human osteonal lamellae.citations
- 2014On the elastic properties of mineralized turkey leg tendon tissue: multiscale model and experiment.citations
- 2014Modeling of femoral neck cortical bone for the numerical simulation of ultrasound propagation.citations
- 2014Ultrasound biomicroscopy (UBM) and scanning acoustic microscopy (SAM) for the assessment of hernia mesh integration: a comparison to standard histology in an experimental model.citations
- 2014Multiscale, Converging Defects of Macro-Porosity, Microstructure and Matrix Mineralization Impact Long Bone Fragility in NF1citations
- 2009Assessment of Microelastic Properties of Bone Using Scanning Acoustic Microscopy: A Face-to-Face Comparison with Nanoindentation
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article
Multiscale, Converging Defects of Macro-Porosity, Microstructure and Matrix Mineralization Impact Long Bone Fragility in NF1
Abstract
Bone fragility due to osteopenia, osteoporosis or debilitating focal skeletal dysplasias is a frequent observation in the Mendelian disease Neurofibromatosis type 1 (NF1). To determine the mechanisms underlying bone fragility in NF1 we analyzed two conditional mouse models, Nf1Prx1 (limb knock-out) and Nf1Col1 (osteoblast specific knock-out), as well as cortical bone samples from individuals with NF1. We examined mouse bone tissue with micro-computed tomography, qualitative and quantitative histology, mechanical tensile analysis, small-angle X-ray scattering (SAXS), energy dispersive X-ray spectroscopy (EDX), and scanning acoustic microscopy (SAM). In cortical bone of Nf1Prx1 mice we detected ectopic blood vessels that were associated with diaphyseal mineralization defects. Defective mineral binding in the proximity of blood vessels was most likely due to impaired bone collagen formation, as these areas were completely devoid of acidic matrix proteins and contained thin collagen fibers. Additionally, we found significantly reduced mechanical strength of the bone material, which was partially caused by increased osteocyte volume. Consistent with these observations, bone samples from individuals with NF1 and tibial dysplasia showed increased osteocyte lacuna volume. Reduced mechanical properties were associated with diminished matrix stiffness, as determined by SAM. In line with these observations, bone tissue from individuals with NF1 and tibial dysplasia showed heterogeneous mineralization and reduced collagen fiber thickness and packaging. Collectively, the data indicate that bone fragility in NF1 tibial dysplasia is partly due to an increased osteocyte-related micro-porosity, hypomineralization, a generalized defect of organic matrix formation, exacerbated in the regions of tensional and bending force integration, and finally persistence of ectopic blood vessels associated with localized macro-porotic bone lesions.