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| ==CRYSTAL STRUCTURE OF A SPARC-COLLAGEN COMPLEX== | | ==Crystal structure of a SPARC-collagen complex== |
| <StructureSection load='2v53' size='340' side='right' caption='[[2v53]], [[Resolution|resolution]] 3.20Å' scene=''> | | <StructureSection load='2v53' size='340' side='right'caption='[[2v53]], [[Resolution|resolution]] 3.20Å' scene=''> |
| == Structural highlights == | | == Structural highlights == |
| <table><tr><td colspan='2'>[[2v53]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2V53 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2V53 FirstGlance]. <br> | | <table><tr><td colspan='2'>[[2v53]] 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=2V53 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2V53 FirstGlance]. <br> |
| </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr> | | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.2Å</td></tr> |
| <tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=HYP:4-HYDROXYPROLINE'>HYP</scene></td></tr> | | <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=HYP:4-HYDROXYPROLINE'>HYP</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> |
| <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1nub|1nub]], [[1bmo|1bmo]], [[1sra|1sra]]</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=2v53 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2v53 OCA], [https://pdbe.org/2v53 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2v53 RCSB], [https://www.ebi.ac.uk/pdbsum/2v53 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2v53 ProSAT]</span></td></tr> |
| <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2v53 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2v53 OCA], [http://pdbe.org/2v53 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2v53 RCSB], [http://www.ebi.ac.uk/pdbsum/2v53 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2v53 ProSAT]</span></td></tr> | |
| </table> | | </table> |
| == Disease ==
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| [[http://www.uniprot.org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:[http://omim.org/entry/130020 130020]]; also known as benign hypermobility syndrome. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS3 is a form of Ehlers-Danlos syndrome characterized by marked joint hyperextensibility without skeletal deformity.<ref>PMID:7833919</ref> Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4) [MIM:[http://omim.org/entry/130050 130050]]. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS4 is the most severe form of the disease. It is characterized by the joint and dermal manifestations as in other forms of the syndrome, characteristic facial features (acrogeria) in most patients, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas.<ref>PMID:1370809</ref> <ref>PMID:8411057</ref> <ref>PMID:2492273</ref> <ref>PMID:7749417</ref> <ref>PMID:1352273</ref> <ref>PMID:2808425</ref> <ref>PMID:1895316</ref> <ref>PMID:1357232</ref> <ref>PMID:1496983</ref> <ref>PMID:8098182</ref> [:]<ref>PMID:7912131</ref> <ref>PMID:8019562</ref> [:]<ref>PMID:8680408</ref> <ref>PMID:8884076</ref> <ref>PMID:9147870</ref> <ref>PMID:8664902</ref> <ref>PMID:8990011</ref> <ref>PMID:9036918</ref> <ref>PMID:9452103</ref> <ref>PMID:10923041</ref> <ref>PMID:10706896</ref> <ref>PMID:11168790</ref> <ref>PMID:12694234</ref> <ref>PMID:12786757</ref> Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA) [MIM:[http://omim.org/entry/100070 100070]]. AAA is a common multifactorial disorder characterized by permanent dilation of the abdominal aorta, usually due to degenerative changes in the aortic wall. Histologically, AAA is characterized by signs of chronic inflammation, destructive remodeling of the extracellular matrix, and depletion of vascular smooth muscle cells.<ref>PMID:8514866</ref> <ref>PMID:2243125</ref> <ref>PMID:2349939</ref>
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| == Function == | | == Function == |
| [[http://www.uniprot.org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Collagen type III occurs in most soft connective tissues along with type I collagen. | | [https://www.uniprot.org/uniprot/SPRC_HUMAN SPRC_HUMAN] Appears to regulate cell growth through interactions with the extracellular matrix and cytokines. Binds calcium and copper, several types of collagen, albumin, thrombospondin, PDGF and cell membranes. There are two calcium binding sites; an acidic domain that binds 5 to 8 Ca(2+) with a low affinity and an EF-hand loop that binds a Ca(2+) ion with a high affinity. |
| <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
| == Publication Abstract from PubMed == | | == Publication Abstract from PubMed == |
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| ==See Also== | | ==See Also== |
| *[[Collagen|Collagen]] | | *[[Collagen 3D structures|Collagen 3D structures]] |
| == References == | | == References == |
| <references/> | | <references/> |
| __TOC__ | | __TOC__ |
| </StructureSection> | | </StructureSection> |
| [[Category: Human]] | | [[Category: Homo sapiens]] |
| [[Category: Bachinger, H P]] | | [[Category: Large Structures]] |
| [[Category: Farndale, R W]] | | [[Category: Bachinger HP]] |
| [[Category: Giudici, C]] | | [[Category: Farndale RW]] |
| [[Category: Hohenester, E]] | | [[Category: Giudici C]] |
| [[Category: Sasaki, T]] | | [[Category: Hohenester E]] |
| [[Category: Basement membrane]]
| | [[Category: Sasaki T]] |
| [[Category: Cell adhesion]]
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| [[Category: Collagen]]
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| [[Category: Extracellular matrix]]
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| [[Category: Glycoprotein]]
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| [[Category: Glycosylated protein]]
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| [[Category: Ion transport]]
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| [[Category: Ionic channel]]
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| [[Category: Secreted]]
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| [[Category: Transport]]
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