NOEV (Figure 2A) has an IC50 of 0.2 μM against human GLB1 and increased the enzyme activity between 1.2- and 5.1-fold in mouse fibroblasts expressing GLB1 carrying GM1-gangliosidosis (p.R201C and p.R201H/p.R457Q) or MPS IVB (p.W273L/p.Y83H) mutations. The gene discussed is GLB1; the disease is GM1 gangliosidosis.