Bone Abstracts

Haematopoietic stem cell (HSC) niches are complex structures located in the trabecular regions of the bone in association with bone-lining osteoblasts (endosteal niches) or with perivascular primitive mesenchymal cells (perivascular niches). These cells provide molecular signals that control HSC fate in terms of self-renewal, proliferation, apoptosis, differentiation, homing, quiescence, etc. Osteoclasts have been implicated in HSC mobilization in response to stress or pharmac...

Introduction: Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis is a rare autosomal recessive renal tubular disease caused by mutations in Claudin 16 and 19 genes, often complicated by progressive renal failure. We describe the clinical and genetic features and management of this condition in three patients at our centre.Case 1: A 3-year-old south Asian boy with consanguinity presented with hypocalcemic seizures. Investigations revealed h...

Existence of inflammatory osteoclasts (iOCLs) contributing to pathological bone resorption associated with inflammatory diseases has been suspected for many years. However, specific markers of iOCLs are lacking, making impossible to establish the contribution of iOCLs to such pathologies. Whereas in steady state OCLs derive from monocytes (MN-OCLs), in inflammatory conditions, they also differentiate from dendritic cells (DC-OCLs). We recently showed that these 2 OCL populatio...

Tissue healing/reconstruction as well as exacerbated inflammatory diseases may benefit from stem cell based therapies. Ex vivo isolated tissue mesenchymal stromal cells (MSCs) displaying multi-potent activity and immune-regulatory functions were long ago proposed as therapeutic cells and already tested in many clinical assays. Nevertheless, their use may be restricted because of the few number that can be recovered from adult tissues, their limited in vitro e...

Chronic inflammatory diseases are characterized by a bone destruction mediated by an increased osteoclast (OCL) activity. OCLs are phagocytic cells arising from the myeloid lineage. Indeed, OCLs derive from monocytes (MN-OCLs) and, in an inflammatory context, they also derive from dendritic cells (DC-OCLs). Despite this origin, their role in the immune responses is still unclear. OCLs in steady state have been reported to act as antigen-presenting cells that activate CD8+...

Tissue healing/reconstruction as well as exacerbated inflammatory diseases may benefit from stem cell based therapies. Ex vivo isolated tissue mesenchymal stromal cells (MSCs) displaying multi-potent activity and immune-regulatory functions were long ago proposed as therapeutic cells and already tested in many clinical assays. Nevertheless, their use may be restricted because of the few number that can be recovered from adult tissues, their limited in vitro e...

Chronic inflammatory diseases are characterized by a bone destruction mediated by an increased osteoclast (OCL) activity. OCLs are phagocytic cells arising from the myeloid lineage. Indeed, OCLs derive from monocytes (MN-OCLs) and, in an inflammatory context, they also derive from dendritic cells (DC-OCLs). Despite this origin, their role in the immune responses is still unclear. OCLs in steady state have been reported to act as antigen-presenting cells that activate CD8+ regu...