Effect of Glucocerebrosidase (GCase) Deficiency in Osteoblasts on Mineralization and Osteoclastogenesis: Implications for Bone Pathology in Gaucher Disease

ROZENFELD, P.A; DELPINO, MV; MUCCI JM

orlando

Congreso; Lysosomal Disease Network-11th Annual WORLD Sympos.; 2015

Lysosomal Disease Network

Bone involvement is the most disabling feature in Gaucher disease but the mechanisms leading to this issue are not well understood yet. In the present study we sought to analyze osteoblast involvement in bone pathology (CBE), an acid -glucocerebrosidase (GCase) inhibitor. After 7, 14 and 21 days of culture collagen deposition, mineralization and PKC activity were evaluated. At the same time points conditioned media (CM) from CBE treated and untreated osteoblast was harvested. The induction of osteoclast differentiation was studied in bone marrow derived osteoclast precursors incubated in the presence of CM. Collagen deposition at 14 and 21 days (p