2w85

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{{Seed}}
 
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[[Image:2w85.png|left|200px]]
 
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==Structure of Pex14 in complex with Pex19==
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The line below this paragraph, containing "STRUCTURE_2w85", creates the "Structure Box" on the page.
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<StructureSection load='2w85' size='340' side='right'caption='[[2w85]], [[NMR_Ensembles_of_Models | 10 NMR models]]' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[2w85]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2W85 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2W85 FirstGlance]. <br>
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</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[2w84|2w84]]</div></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2w85 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2w85 OCA], [https://pdbe.org/2w85 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2w85 RCSB], [https://www.ebi.ac.uk/pdbsum/2w85 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2w85 ProSAT]</span></td></tr>
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{{STRUCTURE_2w85| PDB=2w85 | SCENE= }}
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</table>
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== Disease ==
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[[https://www.uniprot.org/uniprot/PEX19_HUMAN PEX19_HUMAN]] Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:[https://omim.org/entry/614886 614886]]; also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies.<ref>PMID:20683989</ref> Defects in PEX19 are the cause of peroxisome biogenesis disorder 12A (PBD12A) [MIM:[https://omim.org/entry/614886 614886]]. A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and clinically characterized by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life.
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== Function ==
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[[https://www.uniprot.org/uniprot/PEX19_HUMAN PEX19_HUMAN]] Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.<ref>PMID:10051604</ref> <ref>PMID:10704444</ref> <ref>PMID:11259404</ref> <ref>PMID:11883941</ref> <ref>PMID:15007061</ref> <ref>PMID:14709540</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/w8/2w85_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=2w85 ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Protein import into peroxisomes depends on a complex and dynamic network of protein-protein interactions. Pex14 is a central component of the peroxisomal import machinery and binds the soluble receptors Pex5 and Pex19, which have important function in the assembly of peroxisome matrix and membrane, respectively. We show that the N-terminal domain of Pex14, Pex14(N), adopts a three-helical fold. Pex5 and Pex19 ligand helices bind competitively to the same surface in Pex14(N) albeit with opposite directionality. The molecular recognition involves conserved aromatic side chains in the Pex5 WxxxF/Y motif and a newly identified F/YFxxxF sequence in Pex19. The Pex14-Pex5 complex structure reveals molecular details for a critical interaction in docking Pex5 to the peroxisomal membrane. We show that mutations of Pex14 residues located in the Pex5/Pex19 binding region disrupt Pex5 and/or Pex19 binding in vitro. The corresponding full-length Pex14 variants are impaired in peroxisomal membrane localisation in vivo, showing that the molecular interactions mediated by the N-terminal domain modulate peroxisomal targeting of Pex14.
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===STRUCTURE OF PEX14 IN COMPEX WITH PEX19===
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Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19.,Neufeld C, Filipp FV, Simon B, Neuhaus A, Schuller N, David C, Kooshapur H, Madl T, Erdmann R, Schliebs W, Wilmanns M, Sattler M EMBO J. 2009 Mar 18;28(6):745-54. Epub 2009 Feb 5. PMID:19197237<ref>PMID:19197237</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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<div class="pdbe-citations 2w85" style="background-color:#fffaf0;"></div>
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(as it appears on PubMed at http://www.pubmed.gov), where 19197237 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_19197237}}
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__TOC__
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</StructureSection>
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==About this Structure==
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[[Category: Human]]
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2W85 is a 2 chains 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=2W85 OCA].
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[[Category: Large Structures]]
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[[Category: David, C]]
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==Reference==
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[[Category: Erdmann, R]]
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<ref group="xtra">PMID:19197237</ref><references group="xtra"/>
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[[Category: Filipp, F V]]
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[[Category: Homo sapiens]]
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[[Category: Kooshapur, H]]
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[[Category: David, C.]]
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[[Category: Madl, T]]
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[[Category: Erdmann, R.]]
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[[Category: Neufeld, C]]
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[[Category: Filipp, F V.]]
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[[Category: Neuhaus, A]]
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[[Category: Kooshapur, H.]]
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[[Category: Sattler, M]]
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[[Category: Madl, T.]]
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[[Category: Schliebs, W]]
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[[Category: Neufeld, C.]]
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[[Category: Schueller, N]]
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[[Category: Neuhaus, A.]]
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[[Category: Simon, B]]
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[[Category: Sattler, M.]]
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[[Category: Wilmanns, M]]
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[[Category: Schliebs, W.]]
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[[Category: Schueller, N.]]
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[[Category: Simon, B.]]
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[[Category: Wilmanns, M.]]
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[[Category: Alternative splicing]]
[[Category: Alternative splicing]]
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[[Category: Cytoplasm]]
 
[[Category: Lipoprotein]]
[[Category: Lipoprotein]]
[[Category: Membrane]]
[[Category: Membrane]]
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[[Category: Peroxisome import]]
[[Category: Peroxisome import]]
[[Category: Peroxisome targeting signal]]
[[Category: Peroxisome targeting signal]]
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[[Category: Pex14]]
 
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[[Category: Pex19]]
 
[[Category: Phosphoprotein]]
[[Category: Phosphoprotein]]
[[Category: Polymorphism]]
[[Category: Polymorphism]]
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[[Category: Receptor-cargo complex]]
[[Category: Receptor-cargo complex]]
[[Category: Translocation]]
[[Category: Translocation]]
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[[Category: Transport]]
 
[[Category: Zellweger syndrome]]
[[Category: Zellweger syndrome]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Apr 22 10:45:38 2009''
 

Current revision

Structure of Pex14 in complex with Pex19

PDB ID 2w85

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