Bone Abstracts

Introduction: Recent data suggest that lipid metabolism imbalances affect osteoblast and osteoclast function resulting in altered bone mass quality and quantity. Here we investigated the role of apolipoprotein A-I (apoA-I), key-element in HDL biogenesis in the pathogenesis of osteoporosis in mice.Materials and methods: Lumbar vertebrae and femora from apoA-I deficient (ApoA-I−/−) and WT (ApoA-I<s...

Introduction: Recent data suggest that imbalances in lipid metabolism affect the function of both osteoblasts and osteoclasts and thus bone quality. Here we investigated the role of apolipoprotein A-I (ApoA-I), a key-element of HDL biogenesis, in the regulation of cardinal genes/proteins that regulate lipoblasts and osteoblasts in mice.Materials and methods: We used apoA-I deficient (ApoA-I−/−) and wild-type (C57BL/6) mice (10 anim...

Background: Lysinuric Protein Intolerance (LPI) is a rare autosomal recessive metabolic disorder affecting amino acid transport. The condition typically presents at weaning, with recurrent diarrhoea and vomiting especially following protein rich meals. It may have a multisystem clinical presentation including growth and haematological abnormalities and rarely osteoporosis. The diagnosis is based on biochemical findings, including increased urine and reduced plasma concentratio...

Background: Three-dimensional scaffolds for the tissue regeneration are also crucial materials for bone formation. In particular, the scaffolds must provide the space for the cell migration and new bone formation. Collagen is one of the useful scaffolds to reach these performance, however, it is not always a desired biodegradability. Recently, we succeeded in developing low adhesive scaffold type I collagen (LASCol) (patent pending) which has the ability to form fibrils. In th...

Bruck syndrome, a disorder caused by bi-allelic mutations in either PLOD2 or FKBP10, is characterized by flexion contractures and bone fractures and shows strong clinical overlap with the brittle bone disease Osteogenesis Imperfecta. PLOD2 encodes the Lysyl hydroxylase 2 (LH2) enzyme, which is responsible for the hydroxylation of lysine residues in the type-I collagen telopeptides. This hydroxylation directs cross-linking of the collagen fibrils in t...

Introduction: Recessive osteogenesis imperfecta (OI) is caused by mutations in genes encoding proteins involved in post-translational interactions with type I collagen. Types VII–IX OI involve defects in the collagen prolyl 3-hydroxylation complex, which modifies α1(I)Pro986. PPIB encodes CyPB, a complex component with PPIase activity and the major isomerase facilitating collagen folding. We investigated the role of CyPB in collagen post-translational modifications a...

Introduction: The interaction between RANK/RANKL is critical for the regulation of osteoclastogenesis and bone resorption. Inhibition of this interaction helps restore the balance between bone resorption and formation. ALX-0141, a novel biological agent (Nanobody) that specifically targets RANKL, was studied in a Phase I trial to assess the safety, tolerability, immunogenicity and PK after single injection.Methods: Forty-two healthy postmenopausal women ...

Objectives: The interaction between RANK and its ligand RANKL is critical for the regulation of osteoclastogenesis and bone resorption. Inhibition of this interaction helps restore the balance between bone resorption and bone formation. ALX-0141, a novel biological agent (Nanobody) that specifically targets RANKL, was studied in a phase I trial to assess the safety, tolerability, immunogenicity and PK after a single s.c. injection.Methods: Forty-two heal...

Background: Osteogenesis Imperfecta is commonly due to dominant mutations in type I collagen genes, COL1A1 and COL1A2. Recessive forms, which are rarer, are caused instead by mutations in various genes coding for proteins involved in collagen post-translational modifications, folding and secretion. A novel disease locus, SERPINF1, coding for pigment-epithelium-derived-factor (PEDF), a likely key factor in bone deposition and remodelling, has been fou...

Objective: Osteoporotic fracture is the most severe complication of osteoporosis and vertebral column suffer the highest risk. The aim of the part I study was to evaluate the prevalence of vertebral fracture, distribution of prevalent fractures as well the grade of the fractures in community-dwelling elderly in China southern city.Methods: A population of 6142 elderly over 50 years old from four communities in China southern city was gathered voluntarily...