1o7a

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[[Image:1o7a.gif|left|200px]]
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{{Seed}}
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[[Image:1o7a.png|left|200px]]
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{{STRUCTURE_1o7a| PDB=1o7a | SCENE= }}
{{STRUCTURE_1o7a| PDB=1o7a | SCENE= }}
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'''HUMAN BETA-HEXOSAMINIDASE B'''
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===HUMAN BETA-HEXOSAMINIDASE B===
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==Overview==
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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.
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The line below this paragraph, {{ABSTRACT_PUBMED_12706724}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 12706724 is the PubMed ID number.
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{{ABSTRACT_PUBMED_12706724}}
==About this Structure==
==About this Structure==
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[[Category: Sandhoff disease]]
[[Category: Sandhoff disease]]
[[Category: Sphingolipid degradation]]
[[Category: Sphingolipid degradation]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 03:28:22 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Jul 27 15:17:33 2008''

Revision as of 12:17, 27 July 2008

Image:1o7a.png

Template:STRUCTURE 1o7a

HUMAN BETA-HEXOSAMINIDASE B

Template:ABSTRACT PUBMED 12706724

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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