3mk4

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{{Seed}}
 
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[[Image:3mk4.jpg|left|200px]]
 
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==X-Ray structure of human PEX3 in complex with a PEX19 derived peptide==
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The line below this paragraph, containing "STRUCTURE_3mk4", creates the "Structure Box" on the page.
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<StructureSection load='3mk4' size='340' side='right'caption='[[3mk4]], [[Resolution|resolution]] 2.42&Aring;' 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'>[[3mk4]] is a 2 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=3MK4 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3MK4 FirstGlance]. <br>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.42&#8491;</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=3mk4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3mk4 OCA], [https://pdbe.org/3mk4 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3mk4 RCSB], [https://www.ebi.ac.uk/pdbsum/3mk4 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3mk4 ProSAT]</span></td></tr>
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{{STRUCTURE_3mk4| PDB=3mk4 | SCENE= }}
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</table>
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== Disease ==
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===X-Ray structure of human PEX3 in complex with a PEX19 derived peptide===
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[https://www.uniprot.org/uniprot/PEX3_HUMAN PEX3_HUMAN] Defects in PEX3 are the cause of peroxisome biogenesis disorder complementation group 12 (PBD-CG12) [MIM:[https://omim.org/entry/614882 614882]; also known as PBD-CGG. 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:10958759</ref> Defects in PEX3 are a cause of peroxisome biogenesis disorder 10A (PBD10A) [MIM:[https://omim.org/entry/614882 614882]. A fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.<ref>PMID:10848631</ref> <ref>PMID:10958759</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/PEX3_HUMAN PEX3_HUMAN] Involved in peroxisome biosynthesis and integrity. Assembles membrane vesicles before the matrix proteins are translocated. As a docking factor for PEX19, is necessary for the import of peroxisomal membrane proteins in the peroxisomes.<ref>PMID:10848631</ref> <ref>PMID:15007061</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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(as it appears on PubMed at http://www.pubmed.gov), where 20554521 is the PubMed ID number.
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Check<jmol>
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{{ABSTRACT_PUBMED_20554521}}
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/mk/3mk4_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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==About this Structure==
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<text>to colour the structure by Evolutionary Conservation</text>
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3MK4 is a 2 chains structure with 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=3MK4 OCA].
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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=3mk4 ConSurf].
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==Reference==
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<div style="clear:both"></div>
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<ref group="xtra">PMID:20554521</ref><references group="xtra"/>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Dodt, G.]]
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[[Category: Large Structures]]
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[[Category: Schmidt, F.]]
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[[Category: Dodt G]]
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[[Category: Stehle, T.]]
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[[Category: Schmidt F]]
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[[Category: Treiber, N.]]
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[[Category: Stehle T]]
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[[Category: Membrane]]
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[[Category: Treiber N]]
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[[Category: Peroxisome]]
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[[Category: Protein transport]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jun 30 13:49:17 2010''
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Current revision

X-Ray structure of human PEX3 in complex with a PEX19 derived peptide

PDB ID 3mk4

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OCA

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