Fabry:Alpha-galactosidase

Summary

α-Galactosidase A with marked active sites

α-galactosidase A (EC 3.2.1.22) is an enzyme that can catalyze the hydrolysis of glycosides. It is homodimeric with an active site in each chain at the amino acids ASP 170 and ASP 231 (which are highlighted in red in the graphic on the side) and is located in the lysosomes of eukaryotic cells.

The protein has an length of 429 aa in its premature form (includes a signal peptide at N-terminal positions 1 to 31) and in its mature form of 398 aa. The enzyme is encoded by the GLA gene that is located on the X-chromosome and which is associated with the Fabry disease.

References

• EC→PDB
• MetaCyc
• KEGG
• OMIM
• PDB
• Swiss-Prot