1gr3

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[[Image:1gr3.png|left|200px]]
 
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==Structure of the human collagen X NC1 trimer==
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The line below this paragraph, containing "STRUCTURE_1gr3", creates the "Structure Box" on the page.
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<StructureSection load='1gr3' size='340' side='right'caption='[[1gr3]], [[Resolution|resolution]] 2.00&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'>[[1gr3]] is a 1 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=1GR3 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1GR3 FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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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&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=CPS:3-[(3-CHOLAMIDOPROPYL)DIMETHYLAMMONIO]-1-PROPANESULFONATE'>CPS</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene></td></tr>
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{{STRUCTURE_1gr3| PDB=1gr3 | SCENE= }}
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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=1gr3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1gr3 OCA], [https://pdbe.org/1gr3 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1gr3 RCSB], [https://www.ebi.ac.uk/pdbsum/1gr3 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1gr3 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/COAA1_HUMAN COAA1_HUMAN] Defects in COL10A1 are the cause of Schmid type metaphyseal chondrodysplasia (SMCD) [MIM:[https://omim.org/entry/156500 156500]. SMCD is a dominantly inherited disorder of the osseous skeleton. The cardinal features of the phenotype are mild short stature, coxa vara and a waddling gait. Radiography usually shows sclerosis of the ribs, flaring of the metaphyses, and a wide irregular growth plate, especially of the knees. A variant form of SMCD is spondylometaphyseal dysplasia Japanese type. It is characterized by spinal involvement comprising mild platyspondyly, vertebral body abnormalities, and end-plate irregularity.<ref>PMID:8304336</ref> <ref>PMID:8004099</ref> <ref>PMID:7876225</ref> <ref>PMID:7607655</ref> <ref>PMID:8782043</ref> <ref>PMID:9067753</ref> <ref>PMID:9852679</ref> <ref>PMID:15880705</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/COAA1_HUMAN COAA1_HUMAN] Type X collagen is a product of hypertrophic chondrocytes and has been localized to presumptive mineralization zones of hyaline cartilage.
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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/gr/1gr3_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=1gr3 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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Collagen X is expressed specifically in the growth plate of long bones. Its C1q-like C-terminal NC1 domain forms a stable homotrimer and is crucial for collagen X assembly. Mutations in the NC1 domain cause Schmid metaphyseal chondrodysplasia (SMCD). The crystal structure at 2.0 A resolution of the human collagen X NC1 domain reveals an intimate trimeric assembly strengthened by a buried cluster of calcium ions. Three strips of exposed aromatic residues on the surface of NC1 trimer are likely to be involved in the supramolecular assembly of collagen X. Most internal SMCD mutations probably prevent protein folding, whereas mutations of surface residues may affect the collagen X suprastructure in a dominant-negative manner.
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===STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER===
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Insight into Schmid metaphyseal chondrodysplasia from the crystal structure of the collagen X NC1 domain trimer.,Bogin O, Kvansakul M, Rom E, Singer J, Yayon A, Hohenester E Structure. 2002 Feb;10(2):165-73. PMID:11839302<ref>PMID:11839302</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>
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<div class="pdbe-citations 1gr3" style="background-color:#fffaf0;"></div>
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==See Also==
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The line below this paragraph, {{ABSTRACT_PUBMED_11839302}}, adds the Publication Abstract to the page
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*[[Collagen 3D structures|Collagen 3D structures]]
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(as it appears on PubMed at http://www.pubmed.gov), where 11839302 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_11839302}}
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__TOC__
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</StructureSection>
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==About this Structure==
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[[1gr3]] is a 1 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=1GR3 OCA].
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==Reference==
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<ref group="xtra">PMID:11839302</ref><ref group="xtra">PMID:9920912</ref><ref group="xtra">PMID:9707340</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Bogin, O.]]
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[[Category: Large Structures]]
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[[Category: Hohenester, E.]]
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[[Category: Bogin O]]
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[[Category: Kvansakul, M.]]
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[[Category: Hohenester E]]
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[[Category: Rom, E.]]
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[[Category: Kvansakul M]]
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[[Category: Singer, J.]]
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[[Category: Rom E]]
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[[Category: Yayon, A.]]
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[[Category: Singer J]]
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[[Category: Collagen]]
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[[Category: Yayon A]]
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[[Category: Connective tissue]]
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[[Category: Extracellular matrix]]
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Current revision

Structure of the human collagen X NC1 trimer

PDB ID 1gr3

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