Osteocyte Alterations contribute to bone pathology in Gaucher Disease

MUCCI JUAN MARCOS; FERREYRA-COMPAGNUCCI MALENA; CRIVARO ANDREA; BONDAR CONSTANZA; ORMAZABAL MAXIMILIANO; DELPINO MARÍA VICTORIA; ROZENFELD PAULA ADRIANA

Congreso; 12th EWG GD; 2016

The aim of our work was to evaluate the involvement of osteocytes in the bone pathology of Gaucher disease (GD). The study was performed with an in vitro model using the MLO-Y4 osteocyte cell line treated with CBE at different time points. Osteoclastogenic potential of conditioned media (CM) from CBE treated osteocytes was evaluated by osteoclast differentiation assays, and RANKL levels were evaluated by immunofluorescence. Connexin-43 expression was assessed by qPCR and apoptosis was studied by Annexin-V and TUNEL stainings. To study the mechanism on osteoclast differentiation, the apoptotic body fraction of the CM and its supernatant were obtained by centrifugation. Both fractions were used in osteoclastogenesis assays with or without OPG. The CM from CBE treated osteocytes induced higher levels of osteoclast differentiation compared to control CM and higher surface RANKL levels were observed in treated cells Connexin-43 expression diminished with CBE treatment and osteocyte apoptosis was increased (p