Studies conducted on fibroblasts taken from patients with MPS type I and II indicated a drastic reduction in the levels of major chaperone proteins from the Hsp family (Hsp70 in MPS I and II at p < 0.001; Hsp40 in MPS I p < 0.001 and MPS II at p = 0.007), reaching about 15% of those in control cells, as indicated by Western blotting immunodetection analyses (Figure 1A,B and Figure 2A,B) and by fluorescence microscopy (Figure 1C,D and Figure 2C,D). The gene discussed is HSP90B2P; the disease is Scheie syndrome.