Bone Abstracts

Fibrous dysplasia of bone (FD) is a crippling skeletal disease caused by activating mutations (R201C, R201H) of the Gsα gene. We recently generated GsαR201C transgenic mice that develop a FD skeletal phenotype. The analyses of these mice demonstrated that increased bone resorption is one of the main morbidity factors in FD and that RANKL is the major molecular mediator of osteolysis at affected skeletal sites.Ob...

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Fibrous dysplasia (FD) is a benign skeletal disease caused by activating mutations of Gsα. These mutations lead to formation of abnormal and mechanically unsound bone and fibrotic tissue. Clinical sequelae include deformity, fracture, and pain. Studies in bisphosphonates have shown improvement in bone pain and inconsistent effects on FD mineralization; however interpretation has been limited by a lack of controlled trials.Objecti...

Objectives: Intravenous cyclical bisphosphonates are widely used to treat children with moderate to severe osteogenesis imperfecta (OI). They increase bone mineral density (BMD), diminish fracture rates and improve mobility. Bisphosphonates are often discontinued when growth is completed. We aimed to determine if the skeletal gains achieved with bisphosphonates persist after their discontinuation in patients with OI.Methods: We assessed patients with OI ...

Osteoblasts have a high rate of energy consumption during bone formation and bone protein synthesis. Therefore, it is likely that decreased energy production that accompanies aging, could contribute to reduced osteoblast activity, a critical feature of senile osteoporosis, and that this reduction might be counteracted by favoring energy availability. Cells of the osteoblastic lineage generate 40–80% of the requested energy through fatty acid degradation, thus the modulati...

Betaine (BET) is a component of many food. It is an essential osmolyte and a source of methyl groups. BET consumed from food sources and through dietary supplements presents similar bioavailability. BET exerts an antioxidant activity and decreases inflammation states. Dietary supplement with BET are used in many inflammation-connected pathologies, although its mechanism of action is not fully understood. Recent studies have shown that BET stimulates muscle fibers differentiati...