1d5s
From Proteopedia
(New page: 200px<br /> <applet load="1d5s" size="450" color="white" frame="true" align="right" spinBox="true" caption="1d5s, resolution 3.0Å" /> '''CRYSTAL STRUCTURE OF...) |
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| - | [[Image:1d5s.gif|left|200px]]<br /> | + | [[Image:1d5s.gif|left|200px]]<br /><applet load="1d5s" size="350" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="1d5s" size=" | + | |
caption="1d5s, resolution 3.0Å" /> | caption="1d5s, resolution 3.0Å" /> | ||
'''CRYSTAL STRUCTURE OF CLEAVED ANTITRYPSIN POLYMER'''<br /> | '''CRYSTAL STRUCTURE OF CLEAVED ANTITRYPSIN POLYMER'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Alpha1-antitrypsin deficiency, which can lead to both emphysema and liver | + | Alpha1-antitrypsin deficiency, which can lead to both emphysema and liver disease, is a result of the accumulation of alpha1-antitrypsin polymers within the hepatocyte. A wealth of biochemical and biophysical data suggests that alpha1-antitrypsin polymers form via insertion of residues from the reactive center loop of one molecule into the beta-sheet of another. However, this long-standing hypothesis has not been confirmed by direct structural evidence. Here, we describe the first crystallographic evidence of a beta-strand linked polymer form of alpha1-antitrypsin: the crystal structure of a cleaved alpha1-antitrypsin polymer. |
==Disease== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1D5S is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1D5S is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences 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=1D5S OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Protein complex]] | [[Category: Protein complex]] | ||
| - | [[Category: Bonneic, B | + | [[Category: Bonneic, B F.Le.]] |
| - | [[Category: Bottomley, S | + | [[Category: Bottomley, S P.]] |
[[Category: Dai, W.]] | [[Category: Dai, W.]] | ||
| - | [[Category: Dunstone, M | + | [[Category: Dunstone, M A.]] |
| - | [[Category: Feil, S | + | [[Category: Feil, S C.]] |
| - | [[Category: Parker, M | + | [[Category: Parker, M W.]] |
| - | [[Category: Pike, R | + | [[Category: Pike, R N.]] |
[[Category: Rossjohn, J.]] | [[Category: Rossjohn, J.]] | ||
| - | [[Category: Whisstock, J | + | [[Category: Whisstock, J C.]] |
[[Category: a beta sheet polymerisation]] | [[Category: a beta sheet polymerisation]] | ||
[[Category: rcl cleavage]] | [[Category: rcl cleavage]] | ||
[[Category: serpin fold]] | [[Category: serpin fold]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 12:13:17 2008'' |
Revision as of 10:13, 21 February 2008
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CRYSTAL STRUCTURE OF CLEAVED ANTITRYPSIN POLYMER
Contents |
Overview
Alpha1-antitrypsin deficiency, which can lead to both emphysema and liver disease, is a result of the accumulation of alpha1-antitrypsin polymers within the hepatocyte. A wealth of biochemical and biophysical data suggests that alpha1-antitrypsin polymers form via insertion of residues from the reactive center loop of one molecule into the beta-sheet of another. However, this long-standing hypothesis has not been confirmed by direct structural evidence. Here, we describe the first crystallographic evidence of a beta-strand linked polymer form of alpha1-antitrypsin: the crystal structure of a cleaved alpha1-antitrypsin polymer.
Disease
Known diseases associated with this structure: Emphysema OMIM:[107400], Emphysema-cirrhosis OMIM:[107400], Hemorrhagic diathesis due to antithrombin Pittsburgh OMIM:[107400], Pulmonary disease, chronic obstructive, susceptibility to OMIM:[107400]
About this Structure
1D5S is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Cleaved antitrypsin polymers at atomic resolution., Dunstone MA, Dai W, Whisstock JC, Rossjohn J, Pike RN, Feil SC, Le Bonniec BF, Parker MW, Bottomley SP, Protein Sci. 2000 Feb;9(2):417-20. PMID:10716194
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