5j5j

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==Crystal structure of a chimera of human Desmocollin-2 EC1 and human Desmoglein-2 EC2-EC5==
==Crystal structure of a chimera of human Desmocollin-2 EC1 and human Desmoglein-2 EC2-EC5==
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<StructureSection load='5j5j' size='340' side='right' caption='[[5j5j]], [[Resolution|resolution]] 3.29&Aring;' scene=''>
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<StructureSection load='5j5j' size='340' side='right'caption='[[5j5j]], [[Resolution|resolution]] 3.29&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[5j5j]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5J5J OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5J5J FirstGlance]. <br>
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<table><tr><td colspan='2'>[[5j5j]] 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=5J5J OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5J5J FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene>, <scene name='pdbligand=GAL:BETA-D-GALACTOSE'>GAL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr>
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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]] 3.29&#8491;</td></tr>
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<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=5j5j FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5j5j OCA], [http://pdbe.org/5j5j PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5j5j RCSB], [http://www.ebi.ac.uk/pdbsum/5j5j PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5j5j ProSAT]</span></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=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene>, <scene name='pdbligand=GAL:BETA-D-GALACTOSE'>GAL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></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=5j5j FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5j5j OCA], [https://pdbe.org/5j5j PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5j5j RCSB], [https://www.ebi.ac.uk/pdbsum/5j5j PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5j5j ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/DSC2_HUMAN DSC2_HUMAN]] Familial isolated arrhythmogenic ventricular dysplasia, right dominant form;Familial isolated arrhythmogenic ventricular dysplasia, biventricular form;Familial isolated arrhythmogenic ventricular dysplasia, left dominant form. The disease is caused by mutations affecting the gene represented in this entry.
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[https://www.uniprot.org/uniprot/DSC2_HUMAN DSC2_HUMAN] Familial isolated arrhythmogenic ventricular dysplasia, right dominant form;Familial isolated arrhythmogenic ventricular dysplasia, biventricular form;Familial isolated arrhythmogenic ventricular dysplasia, left dominant form. The disease is caused by mutations affecting the gene represented in this entry.[https://www.uniprot.org/uniprot/DSG2_HUMAN DSG2_HUMAN] Defects in DSG2 are the cause of familial arrhythmogenic right ventricular dysplasia type 10 (ARVD10) [MIM:[https://omim.org/entry/610193 610193]; also known as arrhythmogenic right ventricular cardiomyopathy 10 (ARVC10). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall.<ref>PMID:16773573</ref> <ref>PMID:20031617</ref> <ref>PMID:19863551</ref> <ref>PMID:21062920</ref> Genetic variations in DSG2 are the cause of susceptibility to cardiomyopathy dilated type 1BB (CMD1BB) [MIM:[https://omim.org/entry/612877 612877]. A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.<ref>PMID:18678517</ref>
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/DSC2_HUMAN DSC2_HUMAN]] Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion. May contribute to epidermal cell positioning (stratification) by mediating differential adhesiveness between cells that express different isoforms.
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[https://www.uniprot.org/uniprot/DSC2_HUMAN DSC2_HUMAN] Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion. May contribute to epidermal cell positioning (stratification) by mediating differential adhesiveness between cells that express different isoforms.[https://www.uniprot.org/uniprot/DSG2_HUMAN DSG2_HUMAN] Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion.
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<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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==See Also==
==See Also==
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*[[Cadherin|Cadherin]]
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*[[Cadherin 3D structures|Cadherin 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Brasch, J]]
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[[Category: Homo sapiens]]
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[[Category: Harrison, O J]]
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[[Category: Large Structures]]
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[[Category: Shapiro, L]]
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[[Category: Brasch J]]
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[[Category: Cell adhesion]]
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[[Category: Harrison OJ]]
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[[Category: Cell surface]]
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[[Category: Shapiro L]]
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[[Category: Desmosome]]
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[[Category: Extracellular cadherin domain]]
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Current revision

Crystal structure of a chimera of human Desmocollin-2 EC1 and human Desmoglein-2 EC2-EC5

PDB ID 5j5j

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