Fig 1. CBE and isofagomine effectively inhibit purified GBA1 at 1 mM and 1 μM, respectively.
A) Dose response of GBA1 inhibition in a cell free system by CBE and isofagomine. Similar dose response curves are seen with and without sodium taurocholate (NaTc). A representative experiment is shown. B) IC50 values for isofagomine and CBE and % inhibition at 1 mM CBE and 1 μM isofagomine, respectively (mean ± SEM). Summary of data from three independent experiments is shown. C) CBE and isofagomine are not potent inhibitors of recombinant GBA3. A representative experiment is shown. D) CBE (1 mM) and isofagomine (1 μM) effectively inhibits GBA1 but not GBA3, irrespective of whether sodium taurocholate is present. The graph shows a summary of three independent experiments. E) Addition of sodium taurocholate (NaTc) increases activity of GBA1 ~ 5-fold but decreases activity of GBA3 by ~ 50%. Graph depicts a summary of three independent experiments.