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Characterization of recombinant human α-glucosidase (rhGAA) in vitro and analysis of pharmacological chaperones stabilization. (a) rhGAA was stable at pH 5.0 for up to 24 hours while rapidly lost its activity at pH 3.0 and 7.0. (b) Coincubation with 10 mmol/l N-acetylcysteine (NAC) rescued rhGAA activity at pH 7.0 in a dose-dependent manner, and (c) persisted even after 48 hours of incubation. (d) NAC thermally stabilized rhGAA increasing by 10.5 ± 0.5 °C its melting temperature.
