1qmb

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{{Seed}}
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{{STRUCTURE_1qmb| PDB=1qmb | SCENE= }}
{{STRUCTURE_1qmb| PDB=1qmb | SCENE= }}
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'''CLEAVED ALPHA-1-ANTITRYPSIN POLYMER'''
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===CLEAVED ALPHA-1-ANTITRYPSIN POLYMER===
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==Overview==
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The function of the serpins as proteinase inhibitors depends on their ability to insert the cleaved reactive centre loop as the fourth strand in the main A beta-sheet of the molecule upon proteolytic attack at the reactive centre, P1-P1'. This mechanism is vulnerable to mutations which result in inappropriate intra- or intermolecular loop insertion in the absence of cleavage. Intermolecular loop insertion is known as serpin polymerisation and results in a variety of diseases, most notably liver cirrhosis resulting from mutations of the prototypical serpin alpha1-antitrypsin. We present here the 2.6 A structure of a polymer of alpha1-antitrypsin cleaved six residues N-terminal to the reactive centre, P7-P6 (Phe352-Leu353). After self insertion of P14 to P7, intermolecular linkage is affected by insertion of the P6-P3 residues of one molecule into the partially occupied beta-sheet A of another. This results in an infinite, linear polymer which propagates in the crystal along a 2-fold screw axis. These findings provide a framework for understanding the uncleaved alpha1-antitrypsin polymer and fibrillar and amyloid deposition of proteins seen in other conformational diseases, with the ordered array of polymers in the crystal resulting from slow accretion of the cleaved serpin over the period of a year.
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(as it appears on PubMed at http://www.pubmed.gov), where 10543942 is the PubMed ID number.
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{{ABSTRACT_PUBMED_10543942}}
==About this Structure==
==About this Structure==
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[[Category: Polymer]]
[[Category: Polymer]]
[[Category: Serpin]]
[[Category: Serpin]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 06:26:41 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 05:36:36 2008''

Revision as of 02:36, 29 July 2008

Image:1qmb.png

Template:STRUCTURE 1qmb

CLEAVED ALPHA-1-ANTITRYPSIN POLYMER

Template:ABSTRACT PUBMED 10543942

About this Structure

1QMB is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

A 2.6 A structure of a serpin polymer and implications for conformational disease., Huntington JA, Pannu NS, Hazes B, Read RJ, Lomas DA, Carrell RW, J Mol Biol. 1999 Oct 29;293(3):449-55. PMID:10543942

Page seeded by OCA on Tue Jul 29 05:36:36 2008

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