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==Crystal structures of homooligomers of collagen type IV. alpha5NC1== | |||
<StructureSection load='5naz' size='340' side='right' caption='[[5naz]], [[Resolution|resolution]] 1.85Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[5naz]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5NAZ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5NAZ FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=PG4:TETRAETHYLENE+GLYCOL'>PG4</scene></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=5naz FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5naz OCA], [http://pdbe.org/5naz PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5naz RCSB], [http://www.ebi.ac.uk/pdbsum/5naz PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5naz ProSAT]</span></td></tr> | ||
</table> | |||
== Disease == | |||
[[http://www.uniprot.org/uniprot/CO4A5_HUMAN CO4A5_HUMAN]] X-linked diffuse leiomyomatosis - Alport syndrome;X-linked Alport syndrome. The disease is caused by mutations affecting the gene represented in this entry. Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS). | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/CO4A5_HUMAN CO4A5_HUMAN]] Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Casino, P]] | [[Category: Casino, P]] | ||
[[Category: Marina, A]] | [[Category: Marina, A]] | ||
[[Category: Non-collagenous domain of collagen type iv. a principal structural component of basement membrane]] | |||
[[Category: Structural protein]] | |||
Revision as of 10:27, 12 September 2018
Crystal structures of homooligomers of collagen type IV. alpha5NC1Crystal structures of homooligomers of collagen type IV. alpha5NC1
Structural highlights
Disease[CO4A5_HUMAN] X-linked diffuse leiomyomatosis - Alport syndrome;X-linked Alport syndrome. The disease is caused by mutations affecting the gene represented in this entry. Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS). Function[CO4A5_HUMAN] Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. |
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