1ktc
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(New page: 200px<br /><applet load="1ktc" size="450" color="white" frame="true" align="right" spinBox="true" caption="1ktc, resolution 2.40Å" /> '''The Structure of alp...)
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Revision as of 17:43, 20 November 2007
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The Structure of alpha-N-Acetylgalactosaminidase
Overview
In the lysosome, glycosidases degrade glycolipids, glycoproteins, and, oligosaccharides. Mutations in glycosidases cause disorders characterized, by the deposition of undegraded carbohydrates. Schindler and Fabry, diseases are caused by the incomplete degradation of carbohydrates with, terminal alpha-N-acetylgalactosamine and alpha-galactose, respectively., Here we present the X-ray structure of alpha-N-acetylgalactosaminidase, (alpha-NAGAL), the glycosidase that removes alpha-N-acetylgalactosamine, and the structure with bound ligand. The active site residues of, alpha-NAGAL are conserved in the closely related enzyme a-galactosidase A, (alpha-GAL). The structure demonstrates the catalytic mechanisms of both, enzymes and reveals the structural basis of mutations causing Schindler, and Fabry diseases. As alpha-NAGAL and alpha-GAL produce type O "universal, donor" blood from type A and type B blood, the alpha-NAGAL structure will, aid in the engineering of improved enzymes for blood conversion.
About this Structure
1KTC is a Single protein structure of sequence from Gallus gallus with NAG, NGA, SO4, GOL and ACY as ligands. Active as Alpha-N-acetylgalactosaminidase, with EC number 3.2.1.49 Full crystallographic information is available from OCA.
Reference
The 1.9 A structure of alpha-N-acetylgalactosaminidase: molecular basis of glycosidase deficiency diseases., Garman SC, Hannick L, Zhu A, Garboczi DN, Structure. 2002 Mar;10(3):425-34. PMID:12005440
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