Bone Abstracts

Background and methods: Pigment epithelium-derived factor (PEDF) is a potent antiangiogenic factor, ubiquitously expressed and secreted in human tissues. Hypertrophic cartilage and osteoblasts express PEDF that binds to type I collagen and glycosaminoglycans in the extracellular matrix. Two rare forms of osteogenesis imperfecta (OI) with intact collagen synthesis are associated with PEDF deficiency. Histological observations revealed excessive osteoid formation and prolonged m...

Peak bone mass is a major determinant of osteoporosis risk and subsequent fragility fractures in older age. There is a wide range of factors influencing peak bone mass, ranging from those acting very early in life, for example in utero and periconception, to those acting through childhood and adolescence into young adulthood. In this presentation I will give an overview of some overarching themes and principles of relevance to peak bone mass, using specific clinical scenarios ...

The relationship between vitamin D status and lean mass accretion in young children is not well understood.Objective: To explore vitamin D status in relation to lean mass outcomes over 12 mo in children 2–8 years.Methods: This was a secondary analysis of trial data (clinicaltrials.gov: NCT02097160, NCT02387892) in Montreal, Canada. Children consumed their normal vitamin D intake for 6 mo (Apr–Oct 2014, n=39) and ...

Survival of chronic diseases in childhood is often achieved utilizing glucocorticoids. However, the survival comes at a cost to the growing skeleton, resulting in impairment in the acquisition of peak bone mass and is the major etiology of secondary osteoporosis in children. We recently found that preosteoclasts secrete platelet derived growth factor type BB (PDGF-BB) to promote angiogenesis and osteogenesis during both modeling and remodeling. As glucocorticoid therapy affect...

Objectives: Early-onset osteoporosis is characterized by low bone mineral density (BMD) and reduced bone strength since childhood or young adulthood. Although several monogenic forms have already been identified, the spectrum of mutations and genes behind this condition remain inadequately characterized. Furthermore, it is not clear whether genetic factors determine susceptibility to bone fractures in children with normal BMD. In order to further explore the genetic background...

The activation of Wnt signaling pathway is blocked by soluble proteins such as WIF-1, sFRP, Dkk1, and sclerostin, which work by sequestering the ligand (Wnt) or the co-receptor/receptor moiety. Recent advances in developing anti blocking agents such as monoclonal antibodies to the sclerostin and Dkk1 protein have generated significant interest as potentially useful approaches to treat patients that could utilize a rapid gain in bone mineral density in the context of osteoporos...

Purpose: The aim of this study is to determine the chemical information in trabecular bone by Raman spectroscopy in the human distal femur at the microstructural level.Methods: The subchondral trabeculae were obtained from the middle of medial articular surface of the distal femurs in the two cadavers (one is with and the other is without OA). Raman spectroscopy, a non-destructive technique, was employed to determine the chemical information of the trabe...

Transcript therapies, using chemically modified messenger RNAs (cmRNAs), are emerging as safer yet promising substitutes for gene and recombinant protein therapies. However, their applications have been limited due to transient translation and relative low stability of cmRNAs, compared to DNAs. Here we showed that vacuum-dried cmRNA-loaded collagen sponges, so called Transcript-Activated Matrices (TAMs), could serve as depots for sustained cmRNA delivery, providing steady stat...

Arrest defective 1 was originally identified as an acetyltransferase essential for the life-cycle progression in yeasts. Its human orthologue hARD1 has been known to express the enzymatic activity and to acetylate several targets such as HIF-1alpha, MLCK-1, and beta-catenin. Here, whether hARD1 takes part in pre-osteoblast differentiation toward calcium-deposing osteoblast was explored. ALP staining and alizalin red S staining showed that osteoblast differentiation was negativ...

Cherubism is a rare genetic disease (OMIM #118400) characterized by a massive jaw bone osteolysis. This pathology appears around 2–5 years old and in the less severe cases spontaneously regresses after puberty. So far the only treatment available is surgery, often disabling and traumatic. As the cherubism pathophysiology is not yet understood, we carried out a thoroughly characterization of the cherubism granulomas from ten unrelated patients to determine the cells involv...