205948.fig.005
Figure 5: Representative FACE profiles of fluorophore-labeled unsaturated disaccharides (ΔDi) obtained from plasma CS isomers of both controls (lane 2) and Fabry’s patients (lane 3). Lane 1: mixture of commercial standard ΔDi (ΔDi-nonSHA, 2-acetamido-2-deoxy-3-O-(4-deoxy-α-L-threo-hex-4-enepyranosyluronic acid)-4-D-glucose; ΔDi-nonSCS, 2-acetamido-2-deoxy-3-0-(4-deoxy-α-L-threo-hex-4-enepyranosyluronic acid)-4-D-galactose; ΔDi-6S, 2-acetamido-2-deoxy-3-0-(4-deoxy-α-L-threo-hex-4-enepyranosyluronic acid)-6-O-sulpho-D-galactose; ΔDi-4S, 2-acetamido-2-deoxy-3-0-(4-deoxy-α-L-threo-hex-4-enepyranosyluronic acid)-4-O-sulpho-D-galactose; ΔDi-mono2S, 2-acetamido-2-deoxy-3-O-(4-deoxy-2-O-sulpho-α-L-threo-hex-4-enepyranosyluronic acid)-D-galactose; ΔDi-di(4,6)S, 2-acetamido-2-deoxy-3-O-(4-deoxy-α-L-threo-hex-4-enepyranosyluronic acid)-4,6-O-sulpho-D-galactose).