Searchable abstracts of presentations at key conferences on calcified tissues

ba0005cabs.oc3.5 | Oral Communications | ECTS2016

Targeting skeletal metastatic breast cancer with bisphosphonic matrix metalloproteinase-2 inhibitors

Tauro Marilena , Laghezza Antonio , Paolo Tortorella , Lynch Conor

Breast to bone metastasis is a common incurable event during breast cancer progression. Identifying the molecular mechanisms at play is vital for the development of new therapies. Matrix metalloproteinases, such as MMP-2, are overexpressed in the bone metastatic microenvironment. Genetic ablation of MMP-2 demonstrated its importance of in driving osteolytic bone metastatic breast cancer and support the rationale for the development of a highly specific MMP-2 inhibitor for the ...

ba0005p244 | Genetics and Epigenetics | ECTS2016

NBAS is the gene mutated in two patients affected by Acrofrontofacionasal Dysostosis type 1

Palagano Eleonora , Zuccarini Giulia , Mantero Stefano , Conte Daniele , Angius Andrea , Uva Paolo , Prontera Paolo , Vezzoni Paolo , Villa Anna , Merlo Giorgio , Sobacchi Cristina

Acrofrontofacionasal Dysostosis type 1 (AFFND1) is a rare human syndrome (estimated prevalence lower than 1:1,000,000), characterized by bone abnormalities in addition to other multiple congenital anomalies and intellectual disability. Only four AFFND1 families, three of Brazilian and one of Indian origin, have been described so far and an autosomal recessive pattern of inheritance has been suggested. The patients are severely affected: the main clinical features are intellect...

ba0001pp117 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2013

Relationship between bone mineral density, body composition, skin sclerosis, and serum 25(OH) vitamin D levels in systemic sclerosis

Corrado Addolorata , Neve Anna , Marucci Arcangela , Colia Ripalta , Mele Angiola , Cantatore Francesco Paolo

Introduction: Hypovitaminosis D is observed in several rheumatic autoimmune diseases, including systemic sclerosis (SSc); nevertheless, data concerning the possible determinants of reduced serum vitamin D levels in this disease are not fully investigated. The aim of this study is to evaluate the relationship between BMD, body mass composition, skin sclerosis, and serum vitamin D levels in two subsets of SSc patients.Patients and methods: 55 Post-menopaus...

ba0003cc1 | (1) | ECTS2014

Molecular diagnosis of osteopetrotic patients with atypical presentations using traditional approaches and exome sequencing

Palagano Eleonora , Pangrazio Alessandra , Strina Dario , Puddu Alessandro , Oppo Manuela , Valentini Maria , Vezzoni Paolo , Villa Anna , Sobacchi Cristina

Autosomal Recessive Osteopetrosis (ARO) presents early in life with extreme sclerosis of the skeleton, reduction of bone marrow spaces, hepatosplenomegaly, cranial nerves compression and severe growth failure. ARO is often lethal and at present the only therapy is HSCT, which should be performed as soon as possible in order to obtain a major benefit. ARO is genetically heterogeneous and delays in clinical diagnosis sometimes occur, due to its rareness and to the presence of co...

ba0005p121 | Cancer and bone: basic, translational and clinical | ECTS2016

MCT1 as a novel target for the treatment of osteolytic bone metastases

Avnet Sofia , Lemma Silvia , Di Pompo Gemma , Sboarina Martina , Porporato Paolo , Perez-Escuredo Jhudit , Sonveaux Pierre , Baldini Nicola

Bone metastasis (BM) is a dismal complication of cancer, occurring frequently in patients with advanced breast carcinoma. During metastatic progression, carcinoma cells harness osteoclast (OC) activity, promoting osteolysis. To adapt to hypoxia and/or to support proliferation, carcinoma cells adopt primarily glycolysis for energy production, therefore releasing lactic acid in the microenvironment through monocarboxylate transporter 4 (MCT4). Stressed by tumor cells, osteoblast...

ba0002oc21 | Miscellaneous | ICCBH2013

A randomized, double-blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone

Boyce Alison M , Kelly Marilyn H , Brillante Beth A , Kushner Harvey , Wientroub Shlomo , Riminucci Mara , Bianco Paolo , Robey Pamela G , Collins Michael T

Fibrous dysplasia (FD) is a benign skeletal disease caused by activating mutations of Gsα. These mutations lead to formation of abnormal and mechanically unsound bone and fibrotic tissue. Clinical sequelae include deformity, fracture, and pain. Studies in bisphosphonates have shown improvement in bone pain and inconsistent effects on FD mineralization; however interpretation has been limited by a lack of controlled trials.Objecti...

ba0005p152 | Cell biology: osteoblasts and bone formation | ECTS2016

Participation of microRNA-34a/RANKL in the osteogenic potential of the Poly(vinylidene-trifluorethylene)/barium titanate membrane

Lopes Helena Bacha , Ferraz Emanuela Prado , de Almeida Adriana Luisa Goncalves , Abuna Rodrigo Paolo Flores , Hassan Mohammad Quamurul , Rosa Adalberto Luiz , Beloti Marcio Mateus

Barrier membranes have been extensively used in dentistry to prevent soft tissue down-growth into the bone defects and to promote alveolar ridge augmentation. Previous studies of our group showed that the Poly(vinylidene-trifluoroethylene)/barium titanate composite (PVDF) enhances both the in vitro osteoblastic differentiation and the in vivo bone repair compared with a commercially available polytetrafluoroethylene (PTFE) membrane. As bone formation may be regulated ...

ba0005oc4.6 | Catabolism and metabolism | ECTS2016

The myokine Irisin improves bone quality and strength

Colaianni Graziana , Cuscito Concetta , Mongelli Teresa , Pignataro Paolo , Liu Peng , Lu Ping , Mori Giorgio , Brunetti Giacomina , Yuen Tony , Sun Li , Reseland Janne , Colucci Silvia , Zaidi Mone , Cinti Saverio , Grano Maria

Irisin is a hormone-like myokine secreted from skeletal muscle in response to exercise. Considering that an intimate relationship between skeletal muscle and bone has been well established and that physical exercise physiologically stimulates the skeleton strengthening, we explored the involvement of Irisin on bone metabolism.Our findings demonstrated that the myokine Irisin improves cortical bone mass and geometry in vivo, supporting the idea t...

ba0005p203 | Cell biology: osteoclasts and bone resorption | ECTS2016

The role of light (TNFSF14) on bone remodeling

Brunetti Giacomina , Gigante Isabella , Oranger Angela , Colaianni Graziana , Di Benedetto Adriana , Tamma Roberto , Lippo Luciana , Pignataro Paolo , Di Comite Mariasevera , Mori Giorgio , Tamada Koji , Ware Carl , Colucci Silvia , Grano Maria

LIGHT (TNFSF14), expressed by different cells of the immune system, binds two trans-membrane receptors: HVEM and LTβR. It is over-expressed in erosive rheumatoid arthritis and lytic myeloma-bone disease and controversial data have been published on its role osteoclast (OC) formation in vitro. Here, we investigated the role of LIGHT on in vitro murine osteoclastogenesis model and bone phenotype in LIGHT−/− mice. Firstly, we showed that murin...