Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp166 | Cell biology: osteoblasts and bone formation | ECTS2013

Activated protein C increases osteoblast proliferation and BMP2 induced bone formation

Shen Kaitlin , Schindeler Aaron J , Cheng Tegan L , Xue Meilang , Little David G , Jackson Chris J

Introduction: Activated protein C (APC) plays an important role in the cutaneous healing of chronic wounds arising from orthopaedic surgery and has cytoprotective and anti-inflammatory properties which may also assist bone repair. The aim of this study was to examine whether APC could directly influence osteoblasts and increase bone formation in a rodent model.Methods: Proliferation of MG-63 osteoblast-like cells was quantified by MTT assay and direct co...

ba0007p90 | (1) | ICCBH2019

Osteogenesis imperfecta due to FKBP10 mutation- shift from high to low bone turnover

Lenherr-Taube Nina , Harrington Jennifer , Murphy Anne , Filipowski Kornelia , Kin On Wong Andy , Horward Andrew , Sochett Etienne

Background: Osteogenesis imperfecta (OI) due to FKBP10 mutation is a rare variant of OI. FKBP10 encodes for FKBP65, a molecular chaperon that interacts with type I procollagen to prevent premature fibril formation and plays a role in collagen crosslinking. Defects in FKBP65 result in a spectrum of moderate to severe OI with remarkable variability in phenotypes.Presenting problem: The patient is the first child of non-consanguineous Caucasian parents. She...

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 ...

ba0004p156 | (1) | ICCBH2015

Children with coeliac disease on gluten free diet have normal bone mass, geometry and muscle mass

Mackinder M , SC Wong , Tsiountsioura M , Shepherd S , Tellemer E , Kyriakou A , Buchanan E , Edwards C , SF Ahmed , P McGrogan , Gersimidis K

Objective: To evaluate musculoskeletal development using pQCT in children with coeliac disease (CD) on gluten free diet (GFD) compared with age and gender matched healthy controlsMethod: 38 children (18 males) with CD on GFD for a duration of 3.6 years (0.6, 12.5) underwent pQCT at 4%, 38 and 66% tibial sites. Bloods were collected in CD children only. Results reported as median (range).Result: Median TTG was 1.8 IU/l (0.1, 114) wi...

ba0006p146 | (1) | ICCBH2017

Stature and longitudinal growth in glucocorticoid naive boys with Duchenne Muscular Dystrophy

Joseph S , Edwards G , DiMarco M , Abu-Arafeh I , Baxter A , Horrocks I , McWilliams K , Naismith K , Stephen E , Ahmed S F , Wong S C

Background: Previous studies with small number of boys with Duchenne Muscular Dystrophy (DMD) suggest that growth failure occurs in glucocorticoid naïve (GC) boys.Objective: To evaluate height and longitudinal growth in boys with DMD prior to GC.Method: Retrospective evaluation in boys with DMD with height measurements obtained for clinical purposes. Out of the 91 boys currently managed in Scotland, 51 had at least one height ...

ba0003pp170 | Cell biology: osteoclasts and bone resorption | ECTS2014

DBZ, a γ-secretase inhibitor, suppresses bone resorption by inhibiting c-Src activity

Jin Won Jong , Kim Ha-Neui , Kim Hong-Hee , Lee Zang Hee

Tyrosine kinase c-Src plays an important role in actin ring formation and bone resorption activity in osteoclasts. Therefore, c-Src has been targeted for the treatment of osteolytic disorders. In the present study, we investigated anti-resorptive effect of dibenzazepine (DBZ), one of the γ-secretase inhibitors (GSIs), on osteoclast-mediated excessive bone resorption. DBZ did not affect osteoclast differentiation, but disturbed actin ring formation and inhibited osteoclast...

ba0005p223 | Energy metabolism and bone, fat and bone | ECTS2016

Cyclic AMP/protein kinase A signalling downregulates Dlx5 expression via inducing C/Ebpβ in 3T3-L1 preadipocytes

Baek Jeong-Hwa , Lee Hye-Lim , Qadir Abdul , Kim Hyun Jeong , Chung Jin , Woo Kyung Mi , Ryoo Hyun-Mo

Distal-less homeobox 5 (Dlx5) is a transcription factor that enhances osteogenic differentiation of mesenchymal stem cells via upregulating the expression of Runx2 and other osteoblast phenotypic genes. We have previously demonstrated that Dlx5 also downregulates adipogenic differentiation of mesenchymal stem cells and that insulin decreases expression levels of Dlx5 via increasing expression levels of miR-124, a microRNA targeting 3′UTR of Dlx5. However, the mechanism o...

ba0003pp263 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Interval changes in bone mineral density in exercising young women with apparent athlete triad syndrome

To William , Wong Margaret

Objective: To evaluate the bone mineral density (BMD) of the axial and appendicular skeleton in a group of collegiate dance students undergoing intensive training between a 24 months interval and to correlate these changes to the presence or absence of apparent athlete triad syndrome (ATS).Methods: Forty full time collegiate dance students were recruited from a tertiary Performing Arts Institute. All subjects had basic anthropometric measurements, a full...

ba0003pp269 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Comparison of peripheral volumetric bone mineral density, quantitative ultrasound and standard radiological methods for monitoring bone mineral density changes 2 years postpartum

To William , Wong Margaret

Objective: To evaluate the BMD changes around 2 years after delivery using standard dual energy X-ray absorptiometry (DXA) of the lumbar spine and hip, peripheral quantitative computerized tomography (pQCT) of the distal tibia and radius, and quantitative ultrasound (QUS) of the oscalcis.Methods: Consecutive patients with uncomplicated singleton pregnancies were recruited from a general obstetric clinic. Standard BMD measurements of the lumbar spine and ...

ba0006oc22 | (1) | ICCBH2017

Type I collagen C-propeptide cleavage deficiency increases bone mineralization and alters bone cell differentiation

Barnes Aileen , Perosky Joseph , Blouin Stephane , Rajpar M. Helen , Khoury Basma , Weis MaryAnn , Klaushofer Klaus , Roschger Paul , Eyre David , Fratzl-Zelman Nadja , Kozloff Kenneth , Marini Joan

High Bone Mass (HBM) osteogenesis imperfecta (OI) is caused by dominant mutations in the C-propeptide cleavage site of COL1A1 or COL1A2, characterized by bone hypermineralization. To elucidate the role of C-propeptide processing in bone mineralization and development, we generated heterozygous HBM mice with both residues (Ala-Asp) of the COL1A1 cleavage site substituted (Thr-Asn) to prevent processing by BMP1. Two, 6- and 12-month WT and HBM bones were examin...