Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p163 | (1) | ICCBH2015

Whole blood gene expression analysis in idiopathic infantile hypercalcemia due to compound heterozygous mutation in the CYP24A1 gene in an Austrian 4-month-old boy and his family

Hofer Daniela , Zachhuber Verena , Lindheim Lisa , Munzker Julia , Trummer Olivia , Schweighofer Natascha , Ulbing Matthias , Obermayer-Pietsch Barbara

Defects in 24-hydroxylation caused by vitamin D-hydroxylase (CYP24A1) loss-of-function mutations lead to decreased degradation of 1,25(OH)2D and the syndrome of idiopathic infantile hypercalcemia. Affected individuals show increased sensitivity to vitamin D and may develop severe hypercalcemia and hypercalciuria, even with small doses of vitamin D.Presenting: The objective of the study was to investigate the gene expression profile in...

ba0001pp101 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2013

Vasculature and bone: stages of atherosclerosis come along with changes in gene expression levels of calcification regulators

Schweighofer Natascha , Aigelsreiter Ariane , Graf-Rechberger Martina , Hacker Nicole , Kniepeiss Daniela , Stiegler Philipp , Trummer Olivia , Pieber Thomas , Ulbing Matthias , Wagner Doris , Muller Helmut , Obermayer-Pietsch Barbara

Calcification in the vasculature is one of the leading causes of cardiovascular diseases and mortality outcomes. Therefore, the aim of our study was to investigate changes in the gene expression of calcification regulators (CR) in arterial vessels during different stages of atherosclerosis and to document potential corresponding changes in the bone. OPG, RANKL, OPN, MGP, BSP-II and RUNX2 were candidate genes for our study in bone, aorta and arteria ilica externa tissue samples...

ba0003pp383 | Other diseases of bone and mineral metabolism | ECTS2014

MicroRNAs as new biomarkers for monitoring of vascular calcification in CKD patients

Ulbing Matthias , Schweighofer Natascha , Leber Bettina , Lemesch Sandra , Rosenkranz Alexander , Eller Kathrin , Kirsch Alexander , Muller Helmut , Stadlbauer Vanessa , Obermayer-Pietsch Barbara

Introduction: Calcification of vessels, especially media calcification, in combination with bone demineralization and disturbed bone metabolism is abundant in patients suffering from end stage renal disease (ESRD). In this project, we compare biomarkers of calcification with a focus on microRNAs from ESRD patients listed for renal transplantation (RTX) and healthy controls.Methods: Samples are collected from kidney transplant patients. At the same time t...

ba0003pp150 | Cell biology: osteoblasts and bone formation | ECTS2014

PRKG1: A novel regulator of human skeletal (mesenchymal) stem cell differentiation

Jafari Abbas , Siersbaek Majken , Dobbelstein Matthias , Kassen Moustapha

Protein kinases are an important class of regulatory elements and pharmacological targets for treatment of a number of diseases including cancer, heart and lung diseases. However, their role in regulation of the skeletal (mesenchymal) stem cell (MSC) functions and bone formation is not fully understood. Thus, we performed functional screening of human kinome, using three siRNAs for each of the ~700 known kinases represented in the human genome. Activity of alkaline phosphatase...

ba0006p133 | (1) | ICCBH2017

Long term treatment with intravenous pamidronate in two children with severe form of juvenile Paget's disease

Verfurth Katja , Hovel Matthias , Schundeln Michael , Bauer Jens , Grasemann Corinna

Presenting problem: Severe forms of juvenile Paget’s Syndrome (JPD) result in extreme bone turnover, necessitating long-term treatment with anti-resorptive drugs to control for bone pain and modeling of bones.Objective: To report clinical and biochemical effects of intravenous treatment with Pamidronate in two children with severe forms of JPD over a time period of 3 and 9 years, respectively. Treatment was commenced at 12 months and 3 years of age,...

ba0001pp510 | Steroid hormones and receptors | ECTS2013

Glucocorticoids inhibit bone formation independent of miRNA regulation

Liu Peng , Baschant Ulrike , Groth Marco , Baumgart Mario , Platzer Matthias , Jack Hans-Martin , Tuckermann Jan

: Glucocorticoid-induced osteoporosis (GIO) is the most frequent secondary osteoporosis in patients undergoing steroid therapy.Recently we demonstrated that the inhibition of bone formation in GIO is occurring in part via the suppression of autocrine cytokines by the glucocorticoid receptor (GR) monomer in osteoblasts (Cell Metab 11, 517–531). Since emerging evidences indicate that microRNAs (miRNAs) play a critical role in the differentiat...

ba0003pp236 | Osteoporosis: evaluation and imaging | ECTS2014

Improved assessment of vertebral cortex thickness by means of analytical deconvolution of radial bone mineral density distributions

Damm Timo , Pena Jaime , Bastgen Jan , Krause Matthias , Campbell Graeme , Barkmann Reinhard , Gluer Claus-Christian

New treatment agents against osteoporosis may not only lead to an improved trabecular structure, but can probably also strengthen the cortex. To assess this treatment effects by means of QCT, one has to deal with significant partial volume effects observing this very thin, but compact structure. A new method for cortical thickness estimation has been developed using an analytical deconvolution approach. After estimating the point-spread-function (PSF) of a scanner/kernel combi...

ba0005p28 | Bone biomechanics and quality | ECTS2016

Alendronate therapy improves anterior vertebral microstructure in osteoporotic bone facilitating fracture risk reduction

vom Scheidt Annika , Krause Matthias , Puschel Klaus , Amling Michael , Busse Bjorn

Although the fracture risk reduction under bisphosphonate treatment in osteoporosis is clinically well established, it remains understudied why the efficacy of bisphosphonate therapy is higher for the prevention of vertebral fractures compared to other fractures.Our aim was to investigate whether anti-resorptive therapy with alendronate would result in different regional variations of structural indices in the vertebral body. We investigated the microstr...