6mpx
From Proteopedia
(Difference between revisions)
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| - | '''Unreleased structure''' | ||
| - | + | ==Twelve chloride ions induce formation and stabilize the NC1 hexamer of collagen IV assembled from transition state trimers== | |
| - | + | <StructureSection load='6mpx' size='340' side='right'caption='[[6mpx]], [[Resolution|resolution]] 1.90Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[6mpx]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6MPX OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6MPX 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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=P6G:HEXAETHYLENE+GLYCOL'>P6G</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene>, <scene name='pdbligand=PGE:TRIETHYLENE+GLYCOL'>PGE</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</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=6mpx FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6mpx OCA], [http://pdbe.org/6mpx PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6mpx RCSB], [http://www.ebi.ac.uk/pdbsum/6mpx PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6mpx ProSAT]</span></td></tr> |
| - | [[Category: | + | </table> |
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/CO4A1_HUMAN CO4A1_HUMAN]] Defects in COL4A1 are a cause of brain small vessel disease with hemorrhage (BSVDH) [MIM:[http://omim.org/entry/607595 607595]]. Brain small vessel diseases underlie 20 to 30 percent of ischemic strokes and a larger proportion of intracerebral hemorrhages. Inheritance is autosomal dominant.<ref>PMID:16598045</ref> <ref>PMID:17696175</ref> <ref>PMID:17379824</ref> <ref>PMID:20385946</ref> <ref>PMID:19477666</ref> Defects in COL4A1 are the cause of hereditary angiopathy with nephropathy aneurysms and muscle cramps (HANAC) [MIM:[http://omim.org/entry/611773 611773]]. The clinical renal manifestations include hematuria and bilateral large cysts. Histologic analysis revealed complex basement membrane defects in kidney and skin. The systemic angiopathy appears to affect both small vessels and large arteries.<ref>PMID:18160688</ref> <ref>PMID:20818663</ref> Defects in COL4A1 are a cause of familial porencephaly (POREN1) [MIM:[http://omim.org/entry/175780 175780]]. Porencephaly is a term used for any cavitation or cerebrospinal fluid-filled cyst in the brain. Porencephaly type 1 is usually unilateral and results from focal destructive lesions such as fetal vascular occlusion or birth trauma. Type 2, or schizencephalic porencephaly, is usually symmetric and represents a primary defect or arrest in the development of the cerebral ventricles.<ref>PMID:15905400</ref> <ref>PMID:16107487</ref> <ref>PMID:19194877</ref> | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/CO4A1_HUMAN CO4A1_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.<ref>PMID:10811134</ref> <ref>PMID:16481288</ref> <ref>PMID:16151532</ref> <ref>PMID:18775695</ref> Arresten, comprising the C-terminal NC1 domain, inhibits angiogenesis and tumor formation. The C-terminal half is found to possess the anti-angiogenic activity. Specifically inhibits endothelial cell proliferation, migration and tube formation. Inhibits expression of hypoxia-inducible factor 1alpha and ERK1/2 and p38 MAPK activation. Ligand for alpha1/beta1 integrin.<ref>PMID:10811134</ref> <ref>PMID:16481288</ref> <ref>PMID:16151532</ref> <ref>PMID:18775695</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Large Structures]] | ||
[[Category: Bauer, R]] | [[Category: Bauer, R]] | ||
| - | [[Category: Hudson, B | + | [[Category: Boudko, S P]] |
| + | [[Category: Hudson, B G]] | ||
| + | [[Category: Structural protein]] | ||
Revision as of 07:07, 3 April 2019
Twelve chloride ions induce formation and stabilize the NC1 hexamer of collagen IV assembled from transition state trimers
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