Proinflammatory and proosteoclastogenic potential of peripheral blood mononuclear cells from Gaucher patients: implication for bone pathology

MUCCI JM; CUELLO F; KISINOVSKY I; LARROUDE M; DELPINO, MV; ROZENFELD, P.A

Haifa

Congreso; 11th meeting of the European Working Group on Gaucher Disease; 2014

European Working Group on Gaucher Disease

The aim of our work was to evaluate the proinflammatory and proosteoclastogenic environment in circulation of patients with Gaucher disease. The study was performed using PBMC from patients under ERT. Percentage of two different monocyte subsets and the production of proinflammatory cytokines were evaluated by flow cytometry. To evaluate the proosteoclastogenic state PBMC from patients were cultured in the presence of M-CSF and RANKL and osteoclast differentiation and activity were assayed. These experiments were carried out in the presence of Velaglucerase to study the effect of ERT. To evaluate the presence of secreted molecules that induced osteoclast differentiation conditioned media (CM) prepared from patients PBMC was used in osteoclastogenesis induction assays and the involvement of TNF-α and RANKL was evaluated using neutralizing molecules. Percentage of the proinflammatory CD14+CD16+ monocyte subset was significantly higher on patients (p