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1o7a

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[[Image:1o7a.gif|left|200px]]<br /><applet load="1o7a" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1o7a.gif|left|200px]]
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caption="1o7a, resolution 2.25&Aring;" />
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'''HUMAN BETA-HEXOSAMINIDASE B'''<br />
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{{Structure
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|PDB= 1o7a |SIZE=350|CAPTION= <scene name='initialview01'>1o7a</scene>, resolution 2.25&Aring;
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|SITE= <scene name='pdbsite=ABC:N-Glycosylation+Site+3'>ABC</scene>
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|LIGAND= <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=GDL:2-ACETAMIDO-2-DEOXY-D-GLUCONO-1,5-LACTONE'>GDL</scene> and <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>
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|ACTIVITY= [http://en.wikipedia.org/wiki/Beta-N-acetylhexosaminidase Beta-N-acetylhexosaminidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.52 3.2.1.52]
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|GENE=
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}}
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'''HUMAN BETA-HEXOSAMINIDASE B'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1O7A is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=NAG:'>NAG</scene>, <scene name='pdbligand=GDL:'>GDL</scene> and <scene name='pdbligand=EDO:'>EDO</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Beta-N-acetylhexosaminidase Beta-N-acetylhexosaminidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.52 3.2.1.52] Known structural/functional Site: <scene name='pdbsite=ABC:N-Glycosylation+Site+3'>ABC</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1O7A OCA].
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1O7A is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1O7A OCA].
==Reference==
==Reference==
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The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease., Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W, J Mol Biol. 2003 May 2;328(3):669-81. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=12706724 12706724]
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The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease., Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W, J Mol Biol. 2003 May 2;328(3):669-81. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/12706724 12706724]
[[Category: Beta-N-acetylhexosaminidase]]
[[Category: Beta-N-acetylhexosaminidase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: sphingolipid degradation]]
[[Category: sphingolipid degradation]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 14:14:18 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 13:06:43 2008''

Revision as of 11:06, 20 March 2008

Image:1o7a.gif


PDB ID 1o7a

Drag the structure with the mouse to rotate
, resolution 2.25Å
Sites:
Ligands: , and
Activity: Beta-N-acetylhexosaminidase, with EC number 3.2.1.52
Coordinates: save as pdb, mmCIF, xml



HUMAN BETA-HEXOSAMINIDASE B


Contents

Overview

Human lysosomal beta-hexosaminidases are dimeric enzymes composed of alpha and beta-chains, encoded by the genes HEXA and HEXB. They occur in three isoforms, the homodimeric hexosaminidases B (betabeta) and S (alphaalpha), and the heterodimeric hexosaminidase A (alphabeta), where dimerization is required for catalytic activity. Allelic variations in the HEXA and HEXB genes cause the fatal inborn errors of metabolism Tay-Sachs disease and Sandhoff disease, respectively. Here, we present the crystal structure of a complex of human beta-hexosaminidase B with a transition state analogue inhibitor at 2.3A resolution (pdb 1o7a). On the basis of this structure and previous studies on related enzymes, a retaining double-displacement mechanism for glycosyl hydrolysis by beta-hexosaminidase B is proposed. In the dimer structure, which is derived from an analysis of crystal packing, most of the mutations causing late-onset Sandhoff disease reside near the dimer interface and are proposed to interfere with correct dimer formation. The structure reported here is a valid template also for the dimeric structures of beta-hexosaminidase A and S.

Disease

Known diseases associated with this structure: Sandhoff disease, infantile, juvenile, and adult forms OMIM:[606873], Spinal muscular atrophy, juvenile OMIM:[606873]

About this Structure

1O7A is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease., Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W, J Mol Biol. 2003 May 2;328(3):669-81. PMID:12706724

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