Osteoimmunology in Gaucher disease. TNF-α involvement on an in vitro murine model.

MUCCI JM; DE FRANCESCO, PN; DELPINO MV; ROZENFELD PA

Cordoba

Congreso; Reunion anual de la SAI; 2013

SAI

Gaucher disease (GD) is a genetic lysosomal storage disorder caused by the deficiency of the enzyme beta-glucosidase (GBA). Skeletal manifestations in GD include osteopenia and osteonecrosis, and are the most disabling aspect of the disease, causing high morbidity and a significant decrease in quality of life. Inflammation is a key factor in the pathogenesis of GD. Previous results from our group showed an increase in osteclastogenesis in our human in vitro model of the GD with involvement of T cells and TNF- as a soluble mediator. In the present work we aimed to test an in vitro model of GD using mouse cells to correlate our previous findings. We used two sources of cells from monocyte/machrophages lineage isolated from normal mice, splenocytes (S) and peritoneal machrophages (PM), and were exposed to CBE, the inhibitor of GCase (S-CBE and MP-CBE, respectively). Addition of both conditioned media (CM) from S-CBE and PM-CBE induced the differentiation of osteoclasts precursors from bone marrow (BMP) to mature and functional osteoclasts (p