Bone Abstracts

Infantile malignant osteopetrosis (IMO) is a rare, lethal, recessive disorder characterized by dysfunctional osteoclasts. TCIRG1, encoding the osteoclast V-ATPase, is mutated in 50% of IMO patients. We have previously shown that the resorptive function in osteoclasts derived from IMO patients can be restored in vitro by expressing TCIRG1 using a lentiviral vector. In this study, we aim to investigate the cellular response to vector-derived TCIRG1 expression and to det...

Ankylosing spondylitis is a chronic inflammatory rheumatic disease, characterized by axial pain and osteoproliferation, leading to painful rigidity of the spine and disability. In contrast with this bone formation, bone loss is an early event in this disease, and an increased vertebral fracture risk (but not non-vertebral fracture risk) has been reported in these patients.Prospective studies have shown that potent anti-inflammatory drugs, such as anti-TN...

Hypophosphatasia (HPP) is a rare disease characterized by low enzymatic activity of tissue-nonspecific alkaline phosphatase (TNSALP) resulting in an accumulation of its endogenous substrates like pyridoxal phosphate (PLP) and inorganic pyrophosphate (PPi). The ectoenzyme plays an important role during bone mineralization and might contribute to proper function of kidney and muscle. Neurological symptoms of HPP like seizures, anxiety disorders and depression provide ...

Celiac disease (CD) is a frequent cause of malabsortion in childhood and affects calcium and vitamin D absorption.Aim: Analyze the vitamin D levels and bone mineral density (BMD) at diagnosis in patients affected with CD, and the influence of the age of diagnosis in the clinical presentation.Patient: 52 patients (female: 26) were evaluated.Results: The percentage with vitamin D deficiency (<20 ng/ml) was ...

Molecules secreted by the osteoclast or ‘clastokynes’ are essential to stimulate bone formation by osteoblasts. Treatments with bisphosphates and Denosumab target osteoclast survival and differentiation. This suppresses bone turnover and is suspected to increase the risk of atypical fractures in the long term. A solution to overcome this is to develop strategies that target selectively the activity of osteoclasts without affecting their survival or differentiation. S...

It is well known that the epidermal growth factor receptor (EGFR), its ligands, and the structurally related receptor ERBB2/neu are expressed in skeletal cells. However, the functions of EGFR ligands in bone cells remain poorly defined. In this study, we employed a transgenic mouse line overexpressing the EGFR ligand amphiregulin (AREG) specifically in osteoblasts under the α1(I)-collagen promoter. AREG-tg mice did not show changes in body weight or gross phenotype. Compa...

Background and objective: Targeting bone and cartilage includes approaches using tetracyclines, peptide conjugates, as well as anionic moieties like bisphosphonates. However, the selective targeting of the different compartments still remains a challenge. In previous studies native and demineralized ovine bone was used to analyze the affinity of polyanions derived from dendritic polyglycerol (dPG) toward hydroxyapatite and collagen. Whereas the neutral polymer did not show any...

Objective: To investigate i) the analytic stability of PTH in venous blood specimens in serum tubes processed at different time intervals after collection compared to immediately processed blood samples collected in K2EDTA tubes, and ii) whether storage of samples in serum tubes for 5 years at −20 °C had an effect on stability of PTH.Methods: Venous blood samples were collected from ten healthy Caucasian females. One K2EDTA tube and three seru...

Introduction: The purpose of this study was to evaluate whether daily treatment with 400 IU vitamin D3 was sufficient to maintain 25-hydroxyvitamin D (25(OH)D) concentrations above 60 nmol/l over a 3-year period. In addition, the study aimed to clarify if any differences existed in serum 25(OH)D between pre-supplemented women and women who already had serum 25(OH)D above 60 nmol/l at screening.Methods: Serum samples drawn from 251 postmenopaus...

Background: The mineral content of the skeleton in children may be estimated by a number of methods including DXA-scanning, ultrasound, pQCT-scanning and from plain radiographs. Recently, a new method offering an estimating of the bone mineral content in children based on computerised assessment from hand radiographs has been introduced. The new measure is named bone health index (BHI) and expresses the mineral content in the metacarpal bones divided by the volume of the same ...