Searchable abstracts of presentations at key conferences on calcified tissues

ba0002p71 | (1) | ICCBH2013

The microarchitecture of bone in osteochondromas

Staal Heleen , van Rietbergen Bert , van Rhijn Lodewijk

Introduction: Hereditary multiple osteochondromas (HMO) is characterized by the outward growth of cartilage-capped bone tumors. Osteochondromas contain a bone marrow cavity continuous with the normal bone cavity. Because of their off-axis position, osteochondromas are expected to carry less load than normal bone tissue. According to Wolff’s law, we therefore hypothesized that osteochondromas would have a less developed, osteoporotic-like microstructure. To test this hypot...

ba0004op7 | (1) | ICCBH2015

Lessons from homocystinuria: Cystathionine beta-synthase as a novel marker for osteogenic differentiation of human mesenchymal stem cells

Gambari Laura , Manferdini Cristina , Gabusi Elena , Paolella Francesca , Lisignoli Gina , Mariani Erminia , Grassi Francesco

Objective: Classical homocystinuria due to cystathionine beta-synthase (CBS) deficiency, is a rare autosomal recessively inherited disease characterized by the multiple involvement on different organs. While the most striking cause of morbidity and mortality is thromboembolism, patients develop a marked osteoporosis at early age along with many other skeletal abnormalities. As CBS normally converts homocysteine to cystathionine, the result of CBS deficiency is an accumulation ...

ba0005cabs.op1.1 | Oral Poster Talks | ECTS2016

Osteoblastic and osteolytic bone metastases induce divergent angiogenic responses

Hensel Janine , Riether Carsten , Wetterwald Antoinette , Temanni Ramzi , Kruithof-de Julio Marianna , van der Pluijm Gabri , Thalmann George N. , Cecchini Marco G.

Cancer cell growth is dependent on the microenvironmental support. Prostate and mammary cancer (PCa and MCa) cells preferentially metastasize to bone, where they induce either an osteoblastic or osteolytic response. These opposite stromal responses suggest that different types of cancers adopt distinct strategies to hijack the bone marrow/bone stroma for their growth support. However, the molecular cues underlying these divergent responses are largely elusive.<p class="abs...

ba0002is15 | Paediatric cancer and bone: round table | ICCBH2013

Osteogenic complications during and after childhood cancer

van den Heuvel-Eibrink Marry

Childhood cancer has become curable in the majority (>70%) of patients. This is mainly due to rising intensity of treatment, including (combinations of) surgery, chemotherapy, radiotherapy and stem cell transplantation. In addition, intensive international collaboration for rare subgroups, enhanced stratification for treatment regimens and optimised supportive care has contributed to the improved survival of pediatric cancer that was accomplished over the last decades....

ba0005s5.2 | Molecular Clocks | ECTS2016

Good times, bad times: (patho)physiology of diurnal rhythms

van der Horst Gijsbertus (Bert)

Like most organisms, we have developed an internal time keeping system that drives daily rhythms in metabolism, physiology and behavior, and allows us to optimally anticipate to the momentum of the day. At the basis of circadian timekeeping lies an intracellular molecular oscillator in which a set of clock genes cyclically regulate their own expression with an approximate (circa) 24-hour (dies) periodicity. The mammalian circadian system consists of a light-entrainable master ...

ba0006oc26 | (1) | ICCBH2017

A randomized, open-label Phase 2 study of KRN23, an investigational fully human Anti-FGF23 monoclonal antibody, in children with X-linked Hypophosphatemia (XLH)

Hogler Wolfgang , Portale Anthony , Imel Erik , Boot Annemieke , Linglart Agnes , Padidela Raja , van't Hoff William , Whyte Michael , Mao Meng , Skrinar Alison , Martin Javier San , Carpenter Thomas

Objectives: In XLH, FGF23-mediated hypophosphatemia leads to defective bone mineralization and rickets. Investigational product KRN23 binds FGF23 and inhibits its activity. The objective of this Phase 2 study was to evaluate the safety and efficacy of KRN23 in 52 children with XLH (ages 5–12 years, ≤Tanner 2).Methods: Patients were randomized to receive KRN23 biweekly (Q2W) or monthly (Q4W) by SC injection. KRN23 dose was titrated (maximum 2 m...

ba0002p159 | (1) | ICCBH2013

Novel SLC34A3 mutation causing mild hypophosphataemia, hypercalciuria and nephrolithiasis but no clinical or radiological evidence of rickets

Steele Caroline , Bradbury Mark , Mughal M Zulf

Background: Genetic disorders of mineral metabolism causing nephrolithiasis and bone abnormalities are uncommon and have a varied clinical spectrum. Hypophosphataemic rickets with hypercalciuria (HHRH) is a rare autosomal-recessive condition, typically presenting with severe rickets and hypophosphataemia. Milder forms can present with hypercalciuria and nephrolithiasis without bone disease. The underlying pathophysiology is due to mutations in the SLC34A3 gene, which encodes t...

ba0001pp225 | Cell biology: osteoclasts and bone resorption | ECTS2013

Inhibition of lipopolysacharide induced osteoclast formation and bone resorption in vitro and in vivo in mice by cystatin C

Fredrik Stralberg , Lindholm Catharina , Lindstrom Erik , Kasprzykowski Franciszek , Saftig Paul , Abrahamson Magnus , Grubb Anders , Lerner Ulf H

RANKL induced osteoclastogenesis is mediated by several transcription factors such as NF-κB, AP-1 and Nfatc1. We have found that also cysteine proteinases are involved in the signaling pathway downstream RANK. Thus, cystatin C, Z-RLVG-CHN2 (the sequence of which is based upon one of the enzyme inhibitory domains in cystatin C) and the fungal molecule E-64 – inhibit RANKL induced mouse and human osteoclast formation in vitro (Strålberg et ...

ba0003pp205 | Muscle, physical activity and bone | ECTS2014

Volumetric bone mineral density at the distal radius in premenopausal Spanish women and grip strength: role of calcium intake

Moran Jose , Maria L Canal-Macias , Roncero Raul , Lavado-Garcia Jesus M , Calderon-Garcia Julian F , Rey-Sanchez Purificacion , Pedrera-Zamorano Juan D

Peripheral computed tomography (pQCT) can estimate volumetric bone mineral density (vBMD) and distinguish trabecular from cortical bone. Few comprehensive studies have examined correlates of vBMD in Spanish premenopausal women.The aim was to study the association between bone microarchitecture, muscle mass and strength in premenopausal Spanish women and to evaluate the role of calcium intake. vBMD was assessed in 164 premenopausal women at the distal rad...

ba0003w1.3 | Extracellular vesicles: from old to new frontiers | ECTS2014

Extracellular RNAs and cell to cell communication

Nolte-t Hoen Esther

Release of RNA into the extracellular space is a newly identified means of intercellular communication operating in many organisms. Such extracellular RNAs can be released via 50–1000 nm vesicles Almost all cell types produce such extracellular vesicles (EV) and the secretion and proteins/lipids per RNA content is regulated by the producing cell. Besides release of vesicle-enclosed genetic material, cells can also release RNA in association with macromolecular protein com...