1d5s
From Proteopedia
|
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
Page seeded by OCA on Mon Nov 12 16:29:24 2007
