Bone Abstracts

Background: Hypophosphataemic rickets (HPR) is an inherited condition of phosphate wasting associated with abnormal bone biochemistry and features of rickets. Most patients respond to treatment with alfacalcidol and oral phosphate. Raised levels of alkaline phosphatase (ALP) and parathyroid hormone (PTH) generally indicate a need for refining treatment, or poor compliance.Presenting problem: We report the case of a 36 week gestation male infant born to c...

Objectives: Hypophosphatasia (HPP) is a rare metabolic disease caused by loss-of-function mutation(s) in the tissue-nonspecific alkaline phosphatase (TNSALP) gene. Resultant low TNSALP leads to defective skeletal mineralization and diverse complications that may include poor growth, proximal muscle weakness, pain, and compromised physical function in children. Here, we describe the burden of HPP in patients <18 years old as assessed by two surveys specific to HPP symptomol...

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Introduction: Wnt/β-catenin signalling and mechanical loading are able to inhibit adipogenesis and to stimulate osteoblastogenesis of mesenchymal stem cells1,2. The involvement of β-catenin signalling in mechanically induced bone formation has already been shown in vivo using a tibia loading model3. The aim of this study was to investigate the influence of the activation of β-catenin on the osteogenic and adip...

Strontium ranelate (SrRan) is an efficient treatment for osteoporosis, because SrRan both inhibits osteoclasts and stimulates osteoblastic bone formation. We have previously shown that SrRan also affects mouse osteocyte signaling towards osteoclast precursors and mature osteoblasts. This study assessed the effect of SrRan on paracrine signaling from mechanically-stimulated human osteocytes towards mesenchymal stem cells.Human primary bone cells, used as ...

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Dysfunctions of Wnt/β-catenin and estrogen receptor signalling resulted in impaired mechanotransduction and bone loss as in osteoporosis. Previous studies demonstrated the interaction of these pathways in mechanotransduction in vitro and in vivo. In this study, the influence of the activation of estrogen receptor and β-catenin signalling on mechanically induced bone formation was investigated in ovariectomized mice. 12-week-old mice were ovariectomi...

Estrogen deficiency promotes bone loss and skeletal muscle dysfunction. Peroxisome proliferator-activated receptor (PPAR) agonists and high-impact exercise both affect musculoskeletal properties. Whereas PPARδ agonists ameliorate training response in muscle, PPARα agonists exert beneficial effects in bone and muscle. In the present study we investigated the effects of the PPARα agonist fenofibrate and high-impact jumping exercise alone and combined in ovariectom...

One growth factor that potentially plays a role in fracture healing is midkine (Mdk). Mdk is expressed in chondrocytes during bone repair (Ohta et al. 1999) and has been shown to influence bone mass and mechanotransduction (Neunaber et al. 2010, Liedert et al. 2011). The aim of the present study was to evaluate the effects of Mdk-deficiency on bone repair in a standardized mouse femur osteotomy model.Mdk-deficient and wildtype ...

Prostate cancer (PCa) is the most common cancer in males. When patients develop metastasis, no curative therapy is available. To find new therapeutic options, it is crucial to understand how PCa cells induce metastasis. Recently, it was shown that PCa cells secrete small extracellular vesicles (EVs) that can be found in the circulation and in bones. Uptake of EVs by other cells may change their behaviour. We previously identified three miRNAs that were uniquely upregulated in ...