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Figure 1 | Orphanet Journal of Rare Diseases

Figure 1

From: Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study

Figure 1

The active site of human lysosomal alpha galactosidase. A monomer of human lysosomal alpha galactosidase is shown as a ribbon. The pocket with the highest proportion of conserved amino acids includes four groups of amino acids: D92, D93, C142, D170, R227, D231 (in red), completely conserved and associated with mutations not responding to DGJ; Y207 (in blue), not conserved and associated with mutations not responding to DGJ; E203, L206, S297 (in yellow) conserved and not associated with mutations tested with DGJ; W47, Y134, K168 (in green) not conserved and not associated with mutations tested with DGJ. R363, the furthest site from active pocket where responsive mutations, R363C and R363 H, have been observed is shown in purple.

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