1ktb

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|PDB= 1ktb |SIZE=350|CAPTION= <scene name='initialview01'>1ktb</scene>, resolution 1.90&Aring;
|PDB= 1ktb |SIZE=350|CAPTION= <scene name='initialview01'>1ktb</scene>, resolution 1.90&Aring;
|SITE=
|SITE=
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|LIGAND= <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene> and <scene name='pdbligand=ACY:ACETIC ACID'>ACY</scene>
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|LIGAND= <scene name='pdbligand=ACY:ACETIC+ACID'>ACY</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>
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|ACTIVITY= [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]
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|ACTIVITY= <span class='plainlinks'>[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] </span>
|GENE= alpha-NAGA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9031 Gallus gallus])
|GENE= alpha-NAGA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9031 Gallus gallus])
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|DOMAIN=
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|RELATEDENTRY=[[1ktc|1KTC]]
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1ktb FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ktb OCA], [http://www.ebi.ac.uk/pdbsum/1ktb PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1ktb RCSB]</span>
}}
}}
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[[Category: Hannick, L.]]
[[Category: Hannick, L.]]
[[Category: Zhu, A.]]
[[Category: Zhu, A.]]
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[[Category: ACY]]
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[[Category: (beta/alpha)8 barrel]]
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[[Category: GOL]]
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[[Category: glycoprotein]]
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[[Category: NAG]]
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[[Category: SO4]]
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[[Category: glycoprotein; (beta/alpha)8 barrel]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 12:21:53 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 21:53:18 2008''

Revision as of 18:53, 30 March 2008


PDB ID 1ktb

Drag the structure with the mouse to rotate
, resolution 1.90Å
Ligands: , , ,
Gene: alpha-NAGA (Gallus gallus)
Activity: Alpha-N-acetylgalactosaminidase, with EC number 3.2.1.49
Related: 1KTC


Resources: FirstGlance, OCA, PDBsum, RCSB
Coordinates: save as pdb, mmCIF, xml



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. 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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