2wl8: Difference between revisions

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-[[Image:2wl8.png|left|200px]]
-<!--
+==X-ray crystal structure of Pex19p==
-The line below this paragraph, containing "STRUCTURE_2wl8", creates the "Structure Box" on the page.
+<StructureSection load='2wl8' size='340' side='right'caption='[[2wl8]], [[Resolution|resolution]] 2.05&Aring;' scene=''>
-You may change the PDB parameter (which sets the PDB file loaded into the applet)
+== Structural highlights ==
-or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
+<table><tr><td colspan='2'>[[2wl8]] is a 4 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=2WL8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2WL8 FirstGlance]. <br>
-or leave the SCENE parameter empty for the default display.
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.05&#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=2wl8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wl8 OCA], [https://pdbe.org/2wl8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2wl8 RCSB], [https://www.ebi.ac.uk/pdbsum/2wl8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2wl8 ProSAT]</span></td></tr>
-{{STRUCTURE_2wl8|  PDB=2wl8  |  SCENE=  }}
+</table>
+== Disease ==
+[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.
+== Function ==
+[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>
+== Evolutionary Conservation ==
+[[Image:Consurf_key_small.gif|200px|right]]
+Check<jmol>
+  <jmolCheckbox>
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wl/2wl8_consurf.spt"</scriptWhenChecked>
+    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
+    <text>to colour the structure by Evolutionary Conservation</text>
+  </jmolCheckbox>
+</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=2wl8 ConSurf].
+<div style="clear:both"></div>
+<div style="background-color:#fffaf0;">
+== Publication Abstract from PubMed ==
+The protein Pex19p functions as a receptor and chaperone of peroxisomal membrane proteins (PMPs). The crystal structure of the folded C-terminal part of the receptor reveals a globular domain that displays a bundle of three long helices in an antiparallel arrangement. Complementary functional experiments, using a range of truncated Pex19p constructs, show that the structured alpha-helical domain binds PMP-targeting signal (mPTS) sequences with about 10 muM affinity. Removal of a conserved N-terminal helical segment from the mPTS recognition domain impairs the ability for mPTS binding, indicating that it forms part of the mPTS-binding site. Pex19p variants with mutations in the same sequence segment abolish correct cargo import. Our data indicate a divided N-terminal and C-terminal structural arrangement in Pex19p, which is reminiscent of a similar division in the Pex5p receptor, to allow separation of cargo-targeting signal recognition and additional functions.
-===X-RAY CRYSTAL STRUCTURE OF PEX19P===
+The peroxisomal receptor Pex19p forms a helical mPTS recognition domain.,Schueller N, Holton SJ, Fodor K, Milewski M, Konarev P, Stanley WA, Wolf J, Erdmann R, Schliebs W, Song YH, Wilmanns M EMBO J. 2010 Aug 4;29(15):2491-500. Epub 2010 Jun 8. PMID:20531392<ref>PMID:20531392</ref>
+From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-<!--
+</div>
-The line below this paragraph, {{ABSTRACT_PUBMED_20531392}}, adds the Publication Abstract to the page
+<div class="pdbe-citations 2wl8" style="background-color:#fffaf0;"></div>
-(as it appears on PubMed at http://www.pubmed.gov), where 20531392 is the PubMed ID number.
+== References ==
--->
+<references/>
-{{ABSTRACT_PUBMED_20531392}}
+__TOC__
+</StructureSection>
-==About this Structure==
-[[2wl8]] is a 4 chain structure with sequence 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=2WL8 OCA].
-==Reference==
-<ref group="xtra">PMID:020531392</ref><references group="xtra"/>
 [[Category: Homo sapiens]]
-[[Category: Erdmann, R.]]
+[[Category: Large Structures]]
-[[Category: Holton, S J.]]
+[[Category: Erdmann R]]
-[[Category: Konarev, P.]]
+[[Category: Holton SJ]]
-[[Category: Roessle, M.]]
+[[Category: Konarev P]]
-[[Category: Schliebs, W.]]
+[[Category: Roessle M]]
-[[Category: Schueller, N.]]
+[[Category: Schliebs W]]
-[[Category: Song, Y H.]]
+[[Category: Schueller N]]
-[[Category: Stanley, W A.]]
+[[Category: Song YH]]
-[[Category: Wilmanns, M.]]
+[[Category: Stanley WA]]
-[[Category: Biogenesis disorder]]
+[[Category: Wilmanns M]]
-[[Category: Membrane]]
-[[Category: Prenylation]]
-[[Category: Protein transport]]
-[[Category: Zellweger syndrome]]