Bone Abstracts

Background: Hypophosphatasia (HPP) results from inactivating mutation(s) in the gene for tissue non-specific alkaline phosphatase (TNSALP). Substantial improvement has been reported in skeletal mineralization and physical function in patients (pts) with life-threatening perinatal and infantile HPP treated for 48 weaks with asfotase alfa, a bone-targeted recombinant human TNSALP.Objective: To evaluate long-term efficacy and tolerability of asfotase alfa i...

Background: Advancing maternal age at childbirth has been associated with a higher risk of pregnancy complications and with adverse short-term and long-term offspring health outcomes, but little is known about the effects of increasing maternal age on offspring bone acquisition.Objective: To investigate associations of maternal age at childbirth with offspring bone parameters in healthy men at the age of peak bone mass.Methods: We ...

see PP163....

Here we identify the lipolytic enzyme hepatic lipase (HL, encoded by Lipc) as a novel cell-autonomous regulator of osteoblast function. In an unbiased genome-wide expression analysis, we find Lipc – which was formerly thought to be expressed almost exclusively by the liver – to be highly induced upon osteoblast differentiation, as verified by quantitative Taqman analyses of primary osteoblasts in vitro and of bone samples in vivo. ...

Pathophysiological calcification in the vasculature favours cardio- and cerebrovascular diseases. In patients with chronic kidney disease vitamin K metabolites are associated with decreased vascular calcification.We investigated the expression of vitamin K dependent proteins (VKDPs) in vessels and bone to identify differences in expression pattern during atherosclerosis (AS) stages and compare the two tissue profiles.Gene expressio...

Objectives: Activity duration and the daily patterns of activity during childhood and adolescence could contribute to long-term bone health. We examined cross-sectional associations between 11 and 12 year old children’s bone health and (1) durations, (2) patterns, and (3) combined durations and patterns of moderate-vigorous physical activity (MVPA) and sedentary behaviour.Methods: Design: Population-based cross-sectional study nested within...

Osteosarcoma is the most frequent primary bone tumor that develops mainly in young adults. The survival rate at 5 years is below 30% for patients with poor response to treatment or with metastasis.The histones modifications are of critical importance in maintaining the transcription program of both normal and tumor cells. The bromodomain and extra-terminal domain (BET) protein family is an important class of ‘histone reading protein’ capable to...

see PP103....

The vicious cycle established between bone associated tumors and bone resorption is the central problem with therapeutic strategies against primary bone tumors and bone metastasis. The bromodomain and extra-terminal domain (BET) protein family is an important class of ‘histone reading protein’ capable to recognize the N-acetylation of lysine residues on histone tails. BET bromain proteins have recently been described as regulators of MYC expression in various tumors....

Objectives: Hypophosphatasia (HPP) is the rare inherited metabolic disease caused by low tissue nonspecific alkaline phosphatase activity. HPP manifests a wide spectrum of complications, which may include HPP-related rickets and compromised physical function in children.Methods: We report on clinical gait assessments based on archival video recordings of six children with onset of HPP symptoms at ≧6 months and documented HPP-related skeletal abnor...