3hr0

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{{STRUCTURE_3hr0| PDB=3hr0 | SCENE= }}
 
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===Crystal structure of Homo sapiens Conserved Oligomeric Golgi subunit 4===
 
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{{ABSTRACT_PUBMED_19651599}}
 
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==Disease==
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==Crystal structure of Homo sapiens Conserved Oligomeric Golgi subunit 4==
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[[http://www.uniprot.org/uniprot/COG4_HUMAN COG4_HUMAN]] Defects in COG4 are the cause of congenital disorder of glycosylation type 2J (CDG2J) [MIM:[http://omim.org/entry/613489 613489]]. It is a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.<ref>PMID:19651599</ref><ref>PMID:19494034</ref>
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<StructureSection load='3hr0' size='340' side='right'caption='[[3hr0]], [[Resolution|resolution]] 1.90&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3hr0]] 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=3HR0 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3HR0 FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/COG4_HUMAN COG4_HUMAN]] Required for normal Golgi function. Plays a role in SNARE-pin assembly and Golgi-to-ER retrograde transport via its interaction with SCFD1.<ref>PMID:19536132</ref>
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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]] 1.9&#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=3hr0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3hr0 OCA], [https://pdbe.org/3hr0 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3hr0 RCSB], [https://www.ebi.ac.uk/pdbsum/3hr0 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3hr0 ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[3hr0]] is a 2 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=3HR0 OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/COG4_HUMAN COG4_HUMAN] Defects in COG4 are the cause of congenital disorder of glycosylation type 2J (CDG2J) [MIM:[https://omim.org/entry/613489 613489]. It is a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.<ref>PMID:19651599</ref> <ref>PMID:19494034</ref>
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==Reference==
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== Function ==
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<ref group="xtra">PMID:019651599</ref><references group="xtra"/><references/>
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[https://www.uniprot.org/uniprot/COG4_HUMAN COG4_HUMAN] Required for normal Golgi function. Plays a role in SNARE-pin assembly and Golgi-to-ER retrograde transport via its interaction with SCFD1.<ref>PMID:19536132</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/hr/3hr0_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=3hr0 ConSurf].
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<div style="clear:both"></div>
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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: Hughson, F M.]]
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[[Category: Large Structures]]
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[[Category: Jeffrey, P D.]]
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[[Category: Hughson FM]]
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[[Category: Nakamura, A.]]
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[[Category: Jeffrey PD]]
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[[Category: Richardson, B C.]]
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[[Category: Nakamura A]]
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[[Category: Ungar, D.]]
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[[Category: Richardson BC]]
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[[Category: Conserved oligomeric golgi complex]]
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[[Category: Ungar D]]
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[[Category: Exocyst]]
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[[Category: Golgi apparatus]]
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[[Category: Intracellular trafficking]]
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[[Category: Membrane]]
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[[Category: Multisubunit tethering complex]]
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[[Category: Phosphoprotein]]
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[[Category: Protein transport]]
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[[Category: Transport]]
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[[Category: Transport protein]]
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[[Category: Vesicle tethering]]
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Current revision

Crystal structure of Homo sapiens Conserved Oligomeric Golgi subunit 4

PDB ID 3hr0

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