4aej - Revision history

OCA at 05:31, 25 August 2022

OCA — Thu, 25 Aug 2022 05:31:33 GMT

←Older revision		Revision as of 05:31, 25 August 2022
Line 3:		Line 3:
	<StructureSection load='4aej' size='340' side='right'caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>		<StructureSection load='4aej' size='340' side='right'caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4AEJ FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4aej]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4AEJ 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=GOL:GLYCEROL'>GOL</scene></td></tr>	+	</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=GOL:GLYCEROL'>GOL</scene></td></tr>
	<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>		<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ae2\|4ae2]], [[2v53\|2v53]], [[4ak3\|4ak3]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[4ae2\|4ae2]], [[2v53\|2v53]], [[4ak3\|4ak3]]</div></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=4aej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aej OCA], [~~http~~://pdbe.org/4aej PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4aej RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4aej PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4aej ProSAT]</span></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=4aej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aej OCA], [https://pdbe.org/4aej PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4aej RCSB], [https://www.ebi.ac.uk/pdbsum/4aej PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4aej ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[~~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>	+	[[https://www.uniprot.org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:[https://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:[https://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:[https://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>
	== 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/CO3A1_HUMAN CO3A1_HUMAN]] Collagen type III occurs in most soft connective tissues along with type I collagen.
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 24:		Line 24:

	==See Also==		==See Also==
-	*[[Collagen\|Collagen]]	+	*[[Collagen 3D structures\|Collagen 3D structures]]
	== References ==		== References ==
	<references/>		<references/>

OCA at 07:42, 3 April 2019

OCA — Wed, 03 Apr 2019 07:42:33 GMT

←Older revision		Revision as of 07:42, 3 April 2019
Line 30:		Line 30:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Aghajari, N]]		[[Category: Aghajari, N]]
	[[Category: Bourhis, J M]]		[[Category: Bourhis, J M]]

OCA at 09:10, 6 March 2019

OCA — Wed, 06 Mar 2019 09:10:19 GMT

←Older revision		Revision as of 09:10, 6 March 2019
Line 1:		Line 1:

	==Crystal structure of Human fibrillar procollagen type III C- propeptide trimer==		==Crystal structure of Human fibrillar procollagen type III C- propeptide trimer==
-	<StructureSection load='4aej' size='340' side='right' caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>	+	<StructureSection load='4aej' size='340' side='right'caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AEJ FirstGlance]. <br>		<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AEJ FirstGlance]. <br>

OCA at 18:46, 5 August 2016

OCA — Fri, 05 Aug 2016 18:46:20 GMT

←Older revision		Revision as of 18:46, 5 August 2016
Line 1:		Line 1:
		+
	==Crystal structure of Human fibrillar procollagen type III C- propeptide trimer==		==Crystal structure of Human fibrillar procollagen type III C- propeptide trimer==
	<StructureSection load='4aej' size='340' side='right' caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>		<StructureSection load='4aej' size='340' side='right' caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AEJ FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AEJ 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=GOL:GLYCEROL'>GOL</scene></td></tr>		</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=GOL:GLYCEROL'>GOL</scene></td></tr>
	<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>		<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ae2\|4ae2]], [[2v53\|2v53]], [[4ak3\|4ak3]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ae2\|4ae2]], [[2v53\|2v53]], [[4ak3\|4ak3]]</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=4aej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aej OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4aej RCSB], [http://www.ebi.ac.uk/pdbsum/4aej PDBsum]</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=4aej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aej OCA], [http://pdbe.org/4aej PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4aej RCSB], [http://www.ebi.ac.uk/pdbsum/4aej PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4aej ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 20:		Line 21:
	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>		From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
	</div>		</div>
		+	<div class="pdbe-citations 4aej" style="background-color:#fffaf0;"></div>

	==See Also==		==See Also==
Line 27:		Line 29:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Homo sapiens~~]]	+	[[Category: Human]]
	[[Category: Aghajari, N]]		[[Category: Aghajari, N]]
	[[Category: Bourhis, J M]]		[[Category: Bourhis, J M]]

OCA at 09:27, 21 December 2014

OCA — Sun, 21 Dec 2014 09:27:53 GMT

←Older revision		Revision as of 09:27, 21 December 2014
Line 1:		Line 1:
-	~~{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}~~	+	==Crystal structure of Human fibrillar procollagen type III C- propeptide trimer==
-	===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===	+	<StructureSection load='4aej' size='340' side='right' caption='[[4aej]], [[Resolution\|resolution]] 2.21Å' scene=''>
-	~~{{ABSTRACT_PUBMED_23001006}}~~	+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[4aej]] is a 3 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=4AEJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AEJ 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=GOL:GLYCEROL'>GOL</scene></td></tr>
		+	<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ae2\|4ae2]], [[2v53\|2v53]], [[4ak3\|4ak3]]</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=4aej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aej OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4aej RCSB], [http://www.ebi.ac.uk/pdbsum/4aej PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[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>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Collagen type III occurs in most soft connective tissues along with type I collagen.
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	The C propeptides of fibrillar procollagens have crucial roles in tissue growth and repair by controlling both the intracellular assembly of procollagen molecules and the extracellular assembly of collagen fibrils. Mutations in C propeptides are associated with several, often lethal, genetic disorders affecting bone, cartilage, blood vessels and skin. Here we report the crystal structure of a C-propeptide domain from human procollagen III. It reveals an exquisite structural mechanism of chain recognition during intracellular trimerization of the procollagen molecule. It also gives insights into why some types of collagen consist of three identical polypeptide chains, whereas others do not. Finally, the data show striking correlations between the sites of numerous disease-related mutations in different C-propeptide domains and the degree of phenotype severity. The results have broad implications for understanding genetic disorders of connective tissues and designing new therapeutic strategies.

-	~~==Disease==~~	+	Structural basis of fibrillar collagen trimerization and related genetic disorders.,Bourhis JM, Mariano N, Zhao Y, Harlos K, Exposito JY, Jones EY, Moali C, Aghajari N, Hulmes DJ Nat Struct Mol Biol. 2012 Oct;19(10):1031-6. doi: 10.1038/nsmb.2389. Epub 2012, Sep 23. PMID:23001006<ref>PMID:23001006</ref>
-	~~[[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>	+

-	~~==Function==~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~[[http:~~/~~/www~~.~~uniprot~~.~~org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Collagen type III occurs in most soft connective tissues along with type I collagen~~.	+	</div>
-		+
-	~~==About this Structure==~~	+
-	~~[[4aej]] is a 3 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=4AEJ OCA].~~	+

	==See Also==		==See Also==
	*[[Collagen\|Collagen]]		*[[Collagen\|Collagen]]
-		+	== References ==
-	==~~Reference~~==	+	<references/>
-	<references ~~group="xtra"~~/><~~references~~/>	+	__TOC__
		+	</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
-	[[Category: Aghajari, N.]]	+	[[Category: Aghajari, N]]
-	[[Category: Bourhis, J M.]]	+	[[Category: Bourhis, J M]]
-	[[Category: Harlos, K.]]	+	[[Category: Harlos, K]]
-	[[Category: Hulmes, D J.S.]]	+	[[Category: Hulmes, D J.S]]
-	[[Category: Jones, E Y.]]	+	[[Category: Jones, E Y]]
-	[[Category: Mariano, N.]]	+	[[Category: Mariano, N]]
-	[[Category: Moali, C.]]	+	[[Category: Moali, C]]
-	[[Category: Zhao, Y.]]	+	[[Category: Zhao, Y]]
	[[Category: Extracellular]]		[[Category: Extracellular]]
	[[Category: Fibrosis]]		[[Category: Fibrosis]]

OCA at 03:37, 25 March 2013

OCA — Mon, 25 Mar 2013 03:37:02 GMT

←Older revision		Revision as of 03:37, 25 March 2013
Line 1:		Line 1:
-	[[Image:4aej.png\|left\|200px]]
-
	{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}		{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}
-
	===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===		===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===
-
	{{ABSTRACT_PUBMED_23001006}}		{{ABSTRACT_PUBMED_23001006}}
		+
		+	==Disease==
		+	[[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>
		+
		+	==Function==
		+	[[http://www.uniprot.org/uniprot/CO3A1_HUMAN CO3A1_HUMAN]] Collagen type III occurs in most soft connective tissues along with type I collagen.

	==About this Structure==		==About this Structure==
	[[4aej]] is a 3 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=4AEJ OCA].		[[4aej]] is a 3 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=4AEJ OCA].
		+
		+	==See Also==
		+	*[[Collagen\|Collagen]]
		+
		+	==Reference==
		+	<references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Aghajari, N.]]		[[Category: Aghajari, N.]]

OCA at 08:59, 16 January 2013

OCA — Wed, 16 Jan 2013 08:59:06 GMT

←Older revision		Revision as of 08:59, 16 January 2013
Line 1:		Line 1:
-	[[Image:4aej.~~jpg~~\|left\|200px]]	+	[[Image:4aej.png\|left\|200px]]

	{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}		{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}
Line 5:		Line 5:
	===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===		===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===

		+	{{ABSTRACT_PUBMED_23001006}}

	==About this Structure==		==About this Structure==

OCA at 05:19, 12 September 2012

OCA — Wed, 12 Sep 2012 05:19:56 GMT

←Older revision		Revision as of 05:19, 12 September 2012
Line 1:		Line 1:
-	~~'''Unreleased structure'''~~	+	[[Image:4aej.jpg\|left\|200px]]

-	~~The entry~~ 4aej ~~is ON HOLD~~ ~~until Paper Publication~~	+	{{STRUCTURE_4aej\| PDB=4aej \| SCENE= }}

-	~~Authors: Bourhis, J.M., Mariano, N., Zhao, Y., Harlos, K., Jones, E.Y., Moali,~~ C~~., Aghajari, N., Hulmes, D.J.S.~~	+	===Crystal structure of Human fibrillar procollagen type III C- propeptide trimer===

-	~~Description: Crystal~~ structure ~~of Human fibrillar procollagen type III C~~-~~propeptide trimer~~	+
		+	==About this Structure==
		+	[[4aej]] is a 3 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=4AEJ OCA].
		+	[[Category: Homo sapiens]]
		+	[[Category: Aghajari, N.]]
		+	[[Category: Bourhis, J M.]]
		+	[[Category: Harlos, K.]]
		+	[[Category: Hulmes, D J.S.]]
		+	[[Category: Jones, E Y.]]
		+	[[Category: Mariano, N.]]
		+	[[Category: Moali, C.]]
		+	[[Category: Zhao, Y.]]
		+	[[Category: Extracellular]]
		+	[[Category: Fibrosis]]
		+	[[Category: Matrix]]
		+	[[Category: Structural protein]]
		+	[[Category: Trimer]]

OCA at 04:33, 29 August 2012

OCA — Wed, 29 Aug 2012 04:33:22 GMT

←Older revision		Revision as of 04:33, 29 August 2012
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 4aej is ON HOLD	+	The entry 4aej is ON HOLD until Paper Publication

	Authors: Bourhis, J.M., Mariano, N., Zhao, Y., Harlos, K., Jones, E.Y., Moali, C., Aghajari, N., Hulmes, D.J.S.		Authors: Bourhis, J.M., Mariano, N., Zhao, Y., Harlos, K., Jones, E.Y., Moali, C., Aghajari, N., Hulmes, D.J.S.

	Description: Crystal structure of Human fibrillar procollagen type III C-propeptide trimer		Description: Crystal structure of Human fibrillar procollagen type III C-propeptide trimer

OCA: Protected "4aej" [edit=sysop:move=sysop]

OCA — Wed, 18 Jan 2012 10:25:44 GMT

Protected "4aej" [edit=sysop:move=sysop]

←Older revision

Revision as of 10:25, 18 January 2012