1ktb
From Proteopedia
(New page: 200px<br /><applet load="1ktb" size="450" color="white" frame="true" align="right" spinBox="true" caption="1ktb, resolution 1.90Å" /> '''The Structure of alp...) |
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| - | [[Image:1ktb.gif|left|200px]]<br /><applet load="1ktb" size=" | + | [[Image:1ktb.gif|left|200px]]<br /><applet load="1ktb" size="350" color="white" frame="true" align="right" spinBox="true" |
caption="1ktb, resolution 1.90Å" /> | caption="1ktb, resolution 1.90Å" /> | ||
'''The Structure of alpha-N-Acetylgalactosaminidase'''<br /> | '''The Structure of alpha-N-Acetylgalactosaminidase'''<br /> | ||
==Overview== | ==Overview== | ||
| - | In the lysosome, glycosidases degrade glycolipids, glycoproteins, and | + | 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== | ==About this Structure== | ||
| - | 1KTB is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Gallus_gallus Gallus gallus] with NAG, SO4, GOL and ACY as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Alpha-N-acetylgalactosaminidase Alpha-N-acetylgalactosaminidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.49 3.2.1.49] Full crystallographic information is available from [http:// | + | 1KTB is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Gallus_gallus Gallus gallus] with <scene name='pdbligand=NAG:'>NAG</scene>, <scene name='pdbligand=SO4:'>SO4</scene>, <scene name='pdbligand=GOL:'>GOL</scene> and <scene name='pdbligand=ACY:'>ACY</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Alpha-N-acetylgalactosaminidase Alpha-N-acetylgalactosaminidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.49 3.2.1.49] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1KTB OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Gallus gallus]] | [[Category: Gallus gallus]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Garboczi, D | + | [[Category: Garboczi, D N.]] |
| - | [[Category: Garman, S | + | [[Category: Garman, S C.]] |
[[Category: Hannick, L.]] | [[Category: Hannick, L.]] | ||
[[Category: Zhu, A.]] | [[Category: Zhu, A.]] | ||
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[[Category: glycoprotein; (beta/alpha)8 barrel]] | [[Category: glycoprotein; (beta/alpha)8 barrel]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:37:42 2008'' |
Revision as of 11:37, 21 February 2008
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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
1KTB is a Single protein structure of sequence from Gallus gallus with , , and 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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