Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p37 | Bone biomechanics and quality | ECTS2016

Elemental composition of compact human bone correlated with the osteocyte network

Roschger Andreas , Roschger Paul , Repp Felix , Wagermaier Wolfgang , Weinkamer Richard , Klaushofer Klaus , Fratzl Peter

Spatial distributions of major and minor chemical elements are supposed to change during tissue maturation and due to bone diseases. Previous studies suggested that osteocytes have the ability to interact with the bone matrix of their environment. For this interaction osteocytes make use of the big inner surface of the osteocyte lacuno-canalicular network (OLCN) in which they are accommodated. The aim of this study was to quantify spatial correlations between the elemental com...

ba0005p62 | Bone development/growth and fracture repair | ECTS2016

Bcl-2-associated athanogene-1 (BAG-1) regulates chondrocyte and osteoblast development

Greenhough Joanna , Papadakis Emmanouil , Cutress Ramsey , Townsend Paul , Oreffo Richard , Tare Rahul

The co-chaperone, Bcl-2-associated athanogene-1 (BAG-1), is expressed by chondrocytes and osteogenic cells, and interacts via heat shock chaperones (HSC70/HSP70) with diverse proteins such as nuclear hormone receptors to regulate cell proliferation, differentiation and apoptosis. Early embryonic lethality in Bag-1 null mice has limited the investigation of the function of BAG-1 in skeletal development. The present study aimed to elucidate the role of BAG-1 in skeletal...

ba0005p97 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2016

Vitamin D therapy: the effects of race, skin colour and genotype

Sujatha Jaya , Kothandapani Gopal , Evans Lucy , Fatma Gossiel , Walsh Jennifer , Eastell Richard , Bishop Nick

Background: It is well established that Asians have lower levels of vitamin D (25OHD) when compared to Caucasians. Vitamin D binding protein (DBP) levels are also lower in some races than in Caucasians.Objectives: i) To find out whether DBP is lower in Asians; ii) To find out whether free 25OHD is lower in Asians; iii) To find out whether PTH is increased in Asians and interpret this in relation to 25OHD levels and iv) To find out whether the increment i...

ba0005p165 | Cell biology: osteoblasts and bone formation | ECTS2016

Canine osteoblasts from trabecular, cortical and subchondral bone present differences in alkaline phosphatase activity

Perpetuo Ines Pedro , Shah Mittal , Parsons Kevin , Orriss Isabel , Doube Michael , Pitsillides Andrew , Meeson Richard

Hip osteoarthritis is a cause of significant morbidity to people and their canine companions. Medical management is frequently insufficient, leading to surgery to relieve pain and regain mobility. Hip replacements are not without potential complications, including loosening and infection. Currently, there is a focus on uncemented implants to decrease these problems, however these rely on the biology of the femur for osseointegration and long-term stability. It has been previou...

ba0005p166 | Cell biology: osteoblasts and bone formation | ECTS2016

Primary osteoblast culture from domestic dog (Canis lupus familiaris)

Perpetuo Ines Pedro , Meeson Richard , Pitsillides Andrew , Doube Michael , Orriss Isabel

Effects of whole bone scaling on isolated osteoblast behaviour are unknown. With two orders of magnitude range in body mass, dog breeds are well-suited to determine such relationships.Femoral heads from three canine hip replacement surgeries were collected. Bone fragments were washed in PBS+AB/AM, trypsin-digested and incubated in 0.2% collagenase. Cells from resultant supernatant were seeded in DMEM+10% FCS+AB/AM at 37 °C, 5% CO2, g...

ba0006p116 | (1) | ICCBH2017

Classical SATB2-associated syndrome with severe osteoporosis, recurrent bony fractures and tibial bowing

Jacobs Benjamin , Rose Anna M. , Zulf Mughal M. , Keen Richard , Wakeling Emma L.

Background: Mutations in SATB2 have been described in association with a unique phenotype known as SATB2-associated syndrome (SAS). This condition is characterised by severe intellectual disability affecting speech development, behaviour, facial features and dental anomalies. Skeletal features and osteoporosis have been reported in older individuals (aged 15–36), in association with point mutations. We report a 24-year-old man with a SATB2 misse...

ba0006p131 | (1) | ICCBH2017

The treatment of Camurati-Engelmann disease with Losartan: a case report

Moylan Alex , Wakeling Emma L. , Mughal M. Zulf , Keen Richard , Thornton Matt , Peeva Daniela , Jacobs Benjamin

Background: Camurati-Engelmann disease (CED) is a rare bone dysplasia characterised by hyperostosis and sclerosis of the diaphyses of the long bones and skull. It is caused by autosomal dominant gain-of function mutations within TGFB1, which result in increased activity of transforming growth factor β1 (TGF-β1). It typically presents in mid-childhood with bone pain, myopathy and progressive immobility. Evidence for treatment is based on a number of case repo...

ba0006p182 | (1) | ICCBH2017

Osteonecrosis results in significant long term morbidity in patients with acute lymphoblastic leukaemia

Amin Nadia , Feltbower Richard , Kinsey Sally , Vora Ajay , Mushtaq Talat , James Beki

Objectives: To determine the national prevalence, management and long term outcomes of patients who develop osteonecrosis after initiation of treatment for acute lymphoblastic leukaemia (ALL).Methods: The central trials unit for the leukaemia trial UKALL2003 identified patients with reported bone toxicity out of the 3126 patients recruited into the study. Questionnaires were sent to each relevant treatment centre requesting information about each patient...

ba0007oc24 | (1) | ICCBH2019

TA-46, a recombinant soluble FGFR3 receptor for the treatment of achondroplasia, is safe and well-tolerated in healthy volunteers

Collins Samuel , Greig Gerard , Porter Richard , Stavenhagen Jeff , Santarelli Luca , Meyer Christian

Objectives: To assess the safety, tolerability and pharmacokinetic (PK) profile of single and multiple doses of TA-46 administered subcutaneously to healthy volunteers.Methods: This was a double-blind, randomized, placebo-controlled trial in a total of 72 subjects. Cohorts of 8 subjects were randomised to receive either TA-46 or placebo in a 3:1 ratio in single ascending dose (SAD) and multiple ascending dose (MAD) cohorts. SAD doses were 0.3, 1, 3, 10 a...