Bone Abstracts

The bone organ integrates the activity of bone tissue, bone marrow, and blood vessels and the factors ensuring this coordination remain ill defined. Bone sialoprotein (BSP) is with osteopontin (OPN) a member of the small integrin binding ligand N-linked glycoprotein (SIBLING) family, involved in bone formation, hematopoiesis, and angiogenesis. In rodents, bone marrow ablation induces a rapid formation of medullary bone which peaks by ~8 days (d8) and is blunted in BSP...

Caspases are the basic enzymatic components in the apoptotic process. Recently, these molecules seem to participate not only in apoptotic but also in other cellular events, particularly in differentiation. This work aimed to further investigate novel roles of executive caspases such as caspase-7. The investigation is based on our previously published findings showing non-apoptotic roles of caspase-7 in odontogenesis.In this study, we followed the localiz...

Caspase-7 belongs to executive apoptotic and pro-inflammatory caspases. Recent reports point to novel roles of these caspases, particularly in cell differentiation. As we revealed such function of caspase-7 in odontogenesis, we turned our attention to participation of this molecule in formation of hard tissue in general.In this work, both type of bones, intramembranous (mandibular/alveolar bone), and endochondral (long bones) were investigated. Mouse hea...

Introduction: Cherubism is a rare pediatric disease with a maxillofacial localization caused by mutations of the SH3BP2 gene. Pathogenesis is well described in the Sh3bp2 KI mouse model that presents a systemic inflammatory and bone phenotypes maintained by TNFα and due to the presence of hypersensitive myeloid precursors. In human, the disease is usually described as a maxillofacial exclusive disease. The aim of our study was to explore the systemic phe...

Cherubism is a rare genetic disease (OMIM #118400) characterized by a massive jaw bone osteolysis. This pathology appears around 2–5 years old and in the less severe cases spontaneously regresses after puberty. So far the only treatment available is surgery, often disabling and traumatic. As the cherubism pathophysiology is not yet understood, we carried out a thoroughly characterization of the cherubism granulomas from ten unrelated patients to determine the cells involv...

Nacre, or mother of pearl, is a promising natural biocompatible biomaterial consisting of aragonite (97%) and of organics (3%) and capable to increase the cell osteogenic activity. It has been established that osteoarthritic osteoblasts present a mineralization defect and, to date, only a few molecules (vitamin D3 and bone morphogenetic protein2) could improve the mineralization potential of this cell type. In this context, we evaluated the impact of nacreous molecules on the ...

Cherubism is a rare genetic disorder characterized by extensive growth of a bilateral granuloma of the jaws, resulting in facial disfigurement.Histologically, the lesions consist of a fibrotic stroma with osteoclastic-like multinuclear giant cells (MGC). Cherubism is caused by gain-of-function mutations in the SH3BP2 protein. SH3BP2 is an intracellular adaptor protein positively regulating the activity of the nuclear factor of activated T-cells c1 (NFATc...

Nacre is able to induce bone-forming cells mineralization, and gains widespread interest in bone regeneration. While, the osteoinductive compounds are not identified yet. The nacre extract, ethanol soluble matrix (ESM), was proven having the capacity to induce the mineralization of mouse MC3T3-E1 cells and to restore mineralization defect in human osteoarthritic osteoblasts. Being tested on the two cells, ESM could induce the formation of mineralization nodules, identified as ...