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{{STRUCTURE_4cmz|  PDB=4cmz  |  SCENE=  }}
===An intertwined homodimer of the PDZ homology domain of periaxin===


==Disease==
==An intertwined homodimer of the PDZ homology domain of periaxin==
[[http://www.uniprot.org/uniprot/PRAX_HUMAN PRAX_HUMAN]] Dejerine-Sottas syndrome;Charcot-Marie-Tooth disease type 4F. The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.  
<StructureSection load='4cmz' size='340' side='right'caption='[[4cmz]], [[Resolution|resolution]] 2.70&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[4cmz]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CMZ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CMZ FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.7&#8491;</td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4cmz FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cmz OCA], [https://pdbe.org/4cmz PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cmz RCSB], [https://www.ebi.ac.uk/pdbsum/4cmz PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cmz ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/PRAX_HUMAN PRAX_HUMAN] Dejerine-Sottas syndrome;Charcot-Marie-Tooth disease type 4F. The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/PRAX_HUMAN PRAX_HUMAN] Seems to be required for maintenance of peripheral nerve myelin sheath. May have a role in axon-glial interactions, possibly by interacting with the cytoplasmic domains of integral membrane proteins such as myelin-associated glycoprotein in the periaxonal regions of the Schwann cell plasma membrane. May have a role in the early phases of myelin deposition.
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Periaxin (PRX) is an abundant protein in the peripheral nervous system, with an important role in myelination. PRX participates in large molecular complexes, most likely through the interactions of its N-terminal PSD-95/Discs-large/ZO-1 (PDZ)-like domain. We present the crystal structures of the PDZ-like domains from PRX and its homologue AHNAK nucleoprotein 2 (AHNAK2). The unique intertwined, domain-swapped dimers provide a structural basis for the homodimerization of both proteins. The core of the homodimer is formed by a 6-stranded antiparallel beta sheet, with every other strand from a different chain. The AHNAK2 PDZ domain structure contains a bound class III ligand peptide. The binding pocket is preformed, and the peptide-PDZ interactions have unique aspects, including two salt bridges and weak recognition of the peptide C terminus. Tight homodimerization may be central to the scaffolding functions of PRX and AHNAK2 in molecular complexes linking the extracellular matrix to the cytoskeletal network.


==Function==
Periaxin and AHNAK nucleoprotein 2 form intertwined homodimers through domain swapping.,Han H, Kursula P J Biol Chem. 2014 Mar 27. PMID:24675079<ref>PMID:24675079</ref>
[[http://www.uniprot.org/uniprot/PRAX_HUMAN PRAX_HUMAN]] Seems to be required for maintenance of peripheral nerve myelin sheath. May have a role in axon-glial interactions, possibly by interacting with the cytoplasmic domains of integral membrane proteins such as myelin-associated glycoprotein in the periaxonal regions of the Schwann cell plasma membrane. May have a role in the early phases of myelin deposition.  


==About this Structure==
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
[[4cmz]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CMZ OCA].
</div>
[[Category: Han, H.]]
<div class="pdbe-citations 4cmz" style="background-color:#fffaf0;"></div>
[[Category: Kursula, P.]]
== References ==
[[Category: Cell cycle]]
<references/>
[[Category: Domain swapping]]
__TOC__
[[Category: Intertwining]]
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Han H]]
[[Category: Kursula P]]
Retrieved from "https://proteopedia.org/4cmz"