Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp475 | Other diseases of bone and mineral metabolism | ECTS2013

Multidisciplinary studies of ancient calcified tissues: renal stones from mummies

Capulli Mattia , Arrizza Lorenzo , Rucci Nadia , Piperni Sara Gemini , Quaresima Raimondo , Giuffra Valentina , Fornaciari Gino , Teti Anna , Ventura Luca

The renal stones found in the mummies of Pandolfo III Malatesta, Lord of Fano (1370–1427) and an anonymous nobleman from Popoli (XVIII century) were investigated using different techniques. Both specimens were examined with binocular stereomicroscopy (BSM) and scanning electron microscopy (SEM), also with energy dispersive X-ray analysis (EDX). Multiple tiny fragments from surface and inner portions were submitted to X-ray diffraction (XRD) analysis. Subsequently, the cal...

ba0001pp507 | Paediatric bone disease | ECTS2013

Perceived activity capability in children and adolescents with osteogenesis imperfecta

Hagberg Maud , Lowing Kristina , Astrom Eva

Introduction: Osteogenesis imperfecta (OI) is a genetic disorder which mainly affects the collagen in the bone mass with fractures and deformities as the main symptoms. In OI there is a great variation in dysfunction related to the disease. Mobility and activities related to mobility are often most difficult. The objective for this study was to find a relevant, valid and reliable instrument to assess the children’s activity capability.Method and par...

ba0002oc8 | Biology | ICCBH2013

Generation of the first mouse model of autosomal dominant type II osteopetrosis harbouring the pG213R-clc7 mutation

Del Fattore Andrea , Gray Amie , Ichikawa Shoji , Chu Kang , Mohammad Khalid S , Capannolo Marta , Capulli Mattia , Muraca Maurizio , Econs Michael J , Teti Anna , Alam Imranul

Autosomal dominant type II osteopetrosis (ADO2) is a rare osteosclerotic disorder due to heterozygous missense mutations of CLC7 gene encoding the type 7 chloride channel. Our two labs (L’Aquila and Indianapolis) independently generated the first C57 black 6 (B6) mouse model of ADO2 by inserting the pG213R-clc7 mutation. We created pG213R-clc7 KI mice using a gene targeting approach. Homozygous mice showed lack of tooth eruption and died within ...

ba0003pp78 | Bone development/growth and fracture repair | ECTS2014

Effects of TSH and calcitriol on bone metabolism: in vivo and in vitro study

Dumic-Cule Ivo , Rogic Dunja , Jezek Damir , Grgurevic Lovorka , Vukicevic Slobodan

TSH exerts both antiresorptive and anabolic effects on bone remodeling in aged ovariectomized rats and TSH−/− mice, supported by clinical results demonstrating that low TSH level is associated with increased bone loss. To further explore the effect of TSH on bone metabolism we here introduced a rat model with removed thyroid and parathyroid glands to obtain low serum concentrations of thyroid and parathyroid hormone, calcitonin and 1,25(OH)2D<...

ba0003pp116 | Cell biology: osteoblasts and bone formation | ECTS2014

Inhibitory effect of GH on the adipogenic commitment of mesenchymal stromal cells derived from human trabecular bone

Bolamperti Simona , Spinello Alice , Sauer Aisha , GianLuigi Moro , Ometti Marco , Fraschini Gianfranco , Guidobono Francesca , Rubinacci Alessandro , Villa Isabella

Besides its well known effect on longitudinal bone growth, GH plays a role in the maintenance of adult bone mass. As aging progresses GH levels decline, bone mass decreases and mesenchymal precursors show a reduced osteogenic differentiation capacity resulting in an increase in bone marrow adipocytes. We investigated the effect of GH on MSC differentiation and the possible involvement of microRNA in this process. Human MSC derived from trabecular specimens, waste material of o...

ba0003pp136 | Cell biology: osteoblasts and bone formation | ECTS2014

Transcriptional profile of osteoblastic cells cultured on titanium surfaces modified by oxidative nanopatterning

Ferreira Maidy , Variola Fabio , Nanci Antonio , Dernowsek Janaina , Passos Geraldo , Bombonato-Prado Karina

Titanium implants have been extensively used in dentistry as a replacement of absent dental elements. The biocompatibility of a material depends on cellular response in contact with a surface. The microarray technology is a tool to obtain an overview of the cell state in terms of large-scale transcriptional expression in different situations. Thus, the objective of this investigation was to evaluate the transcriptional profile of osteoblastic cells from human alveolar bone cul...

ba0003pp266 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Adipose tissue expression of adipocytes and osteoporosis in COPD

Kochetkova Evgenia , Ugay Ludmila , Maistrovskaia Yulia

Aim: Aim of our study was to investigate the serum adiponectin, leptin, and osteoprotegerin (OPG) levels and its expressions in the adipose tissue, and their relationships with bone metabolism and in patients with severe C chronic obstructive pulmonary disease (COPD).Materials and methods: Serum leptin, adiponectin, OPG, the receptor activator of nuclear factor-kB ligand (RANKL), and bone turnover markers (osteocalcin and type 1 collagen C-telopeptide (C...

ba0003pp369 | Other diseases of bone and mineral metabolism | ECTS2014

A homozygous 20 bp intronic deletion in front of exon 8 of the ALPL-gene causes infantile hypophosphatasia: a functional characterization

Mentrup Birgit , Girschick Hermann , Jakob Franz , Hofmann Christine

Mutations of the ALPL-gene are closely related to hypophosphatasia (HPP), an inherited disorder of bone and mineral metabolism with clinically heterogeneous symptoms. To date 278 different mutations have been described, leading to reduction or completely loss of enzymatic activity of the tissue nonspecific alkaline phosphatase (TNAP).We present the case of a 6-year-old boy with clinical features and laboratory results consistent with infantile H...

ba0003pp393 | Other diseases of bone and mineral metabolism | ECTS2014

Adult Niemann–Pick disease type B with myositis ossificans: a case report

Shumnalieva Russka , Monov Simeon , Shoumnalieva-Ivanova Viara , Rashkov Rasho , Stoilov Rumen

Introduction: Niemann–Pick disease (NPD) is a rare autosomal recessive lysosomal lipid storage disorder. It is caused by mutations of genes which products are involved in the metabolism of sphingolipids. Their dysfunction causes sphingomyelin to accumulate in different organs which leads to progressive multisystemic disorder. Types A and B NPD are caused by mutations in sphingomyelin phosphodiesterase-1 gene with deficiency of acid sphingomyelinase (ASM). Types C and D NP...

ba0005p161 | Cell biology: osteoblasts and bone formation | ECTS2016

Vaspin regulates the osteogenic differentiation of MC3T3-E1 through the PI3K-Akt/miR-34c loop

Liu Yuan , Xu Feng , Pei Hong-Xia , Yuan Ling-Qing

Vaspin (visceral adipose tissue-derived serine protease inhibitor) is a newly discovered adipokine that widely participates in diabetes mellitus, polycystic ovarian syndrome and other disorders of metabolism. However, the effect of vaspin on the regulation of osteogenesis and the mechanism responsible are still unclear. Here, we found that vaspin could attenuate the ALP activities, osteocalcin secretion and Runx2 expression in the preosteoblast cell line MC3T3-E1 in a dose-dep...