4cmz

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Template:STRUCTURE 4cmz

Contents 1 An intertwined homodimer of the PDZ homology domain of periaxin 2 Disease 3 Function 4 About this Structure 5 Reference

An intertwined homodimer of the PDZ homology domain of periaxin

Template:ABSTRACT PUBMED 24675079

Disease

[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

[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

4cmz is a 3 chain structure. Full crystallographic information is available from OCA.

Reference

• Han H, Kursula P. Periaxin and AHNAK nucleoprotein 2 form intertwined homodimers through domain swapping. J Biol Chem. 2014 Mar 27. PMID:24675079 doi:http://dx.doi.org/10.1074/jbc.M114.554816