Fig 2: Plasma gpNMB in type 1 GD patients. (A) Western blot detection of gpNMB in 0.5 µL type 1 GD and control plasma. (B) ELISA quantification of gpNMB in type 1 GD (squares) and control plasma (circles). (C) Correlation of plasma gpNMB and glucosylceramide (GlcCer). (D) Correlation of plasma gpNMB and glucosylsphingosine (Glc-Spho). (E) Correlation of plasma gpNMB and chitotriosidase. (F) Correlation of plasma gpNMB and CCL18.

Fig 3: Increased gpNMB in GD spleen. (A) Left panel: northern blot for gpNMB RNA in type 1 GD and control spleen. Right panel: qPCR of gpNMB mRNA in type 1 GD and normal spleen. (B) Western blot detection of gpNMB and chitotriosidase in type 1 GD and control spleen. (C) Western blot detection of gpNMB in human monocyte-derived macrophages (from left to right marker, monocytes and 1, 3, 5, 7, or, 10 days matured macrophages). (D) Immunohistochemical detection of gpNMB in Gaucher cells in type 1 GD spleen using a mouse monoclonal antibody.

Fig 4: Correction of elevated plasma gpNMB by enzyme replacement therapy. (A) ERT-induced reduction of plasma gpNMB (western blot). (B) ERT-induced reductions of plasma gpNMB (ELISA). (C) Correlation of relative change in excess plasma gpNMB and chitotriosidase.