6qu9

<table><tr><td colspan='2'>[[6qu9]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6QU9 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6QU9 FirstGlance]. <br>

</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>

<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6qu9 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6qu9 OCA], [http://pdbe.org/6qu9 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6qu9 RCSB], [http://www.ebi.ac.uk/pdbsum/6qu9 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6qu9 ProSAT]</span></td></tr>

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== Publication Abstract from PubMed ==

The serpinopathies are among a diverse set of conformational diseases that involve the aberrant self-association of proteins into ordered aggregates. alpha1-Antitrypsin deficiency is the archetypal serpinopathy and results from the formation and deposition of mutant forms of alpha1-antitrypsin as "polymer" chains in liver tissue. No detailed structural analysis has been performed of this material. Moreover, there is little information on the relevance of well-studied artificially induced polymers to these disease-associated molecules. We have isolated polymers from the liver tissue of Z alpha1-antitrypsin homozygotes (E342K) who have undergone transplantation, labeled them using a Fab fragment, and performed single-particle analysis of negative-stain electron micrographs. The data show structural equivalence between heat-induced and ex vivo polymers and that the intersubunit linkage is best explained by a carboxyl-terminal domain swap between molecules of alpha1-antitrypsin.

 

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== References ==

[[Category: Heyer-Chauhan, N]]

[[Category: Irving, J A]]

[[Category: Jagger, A M]]

[[Category: Lomas, D A]]

[[Category: Z variant]]