Consistent with our findings, Mu et al. showed that MG-132 could increase residual glucocerebrosidase enzyme activity four-fold in cell lines containing the L444P variant associated with Gaucher disease, and that a combination of MG-132 and a chemical chaperone, 2-Acetamido-2-deoxynojirimycin, could increase residual activity five-fold in cells containing the Tay-Sachs disease causing α-hexosaminidase G269S mutation [27]. This evidence concerns the gene GBA1 and Tay-Sachs disease.