2bz6: Difference between revisions
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== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[2bz6]] is a 2 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=2BZ6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2BZ6 FirstGlance]. <br> | <table><tr><td colspan='2'>[[2bz6]] is a 2 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=2BZ6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2BZ6 FirstGlance]. <br> | ||
</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=346:(R)-(4-CARBAMIMIDOYL-PHENYLAMINO)-[5-ETHOXY-2-FLUORO-3-[(R)-TETRAHYDRO-FURAN-3-YLOXY]-PHENYL]-ACETIC+ACID'>346</scene>, <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>< | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=346:(R)-(4-CARBAMIMIDOYL-PHENYLAMINO)-[5-ETHOXY-2-FLUORO-3-[(R)-TETRAHYDRO-FURAN-3-YLOXY]-PHENYL]-ACETIC+ACID'>346</scene>, <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> | ||
<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1bf9|1bf9]], [[1cvw|1cvw]], [[1dan|1dan]], [[1dva|1dva]], [[1f7e|1f7e]], [[1f7m|1f7m]], [[1fak|1fak]], [[1ff7|1ff7]], [[1ffm|1ffm]], [[1j9c|1j9c]], [[1jbu|1jbu]], [[1kli|1kli]], [[1klj|1klj]], [[1nl8|1nl8]], [[1o5d|1o5d]], [[1qfk|1qfk]], [[1w0y|1w0y]], [[1w2k|1w2k]], [[1w7x|1w7x]], [[1w8b|1w8b]], [[1ygc|1ygc]], [[1z6j|1z6j]]</td></tr> | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1bf9|1bf9]], [[1cvw|1cvw]], [[1dan|1dan]], [[1dva|1dva]], [[1f7e|1f7e]], [[1f7m|1f7m]], [[1fak|1fak]], [[1ff7|1ff7]], [[1ffm|1ffm]], [[1j9c|1j9c]], [[1jbu|1jbu]], [[1kli|1kli]], [[1klj|1klj]], [[1nl8|1nl8]], [[1o5d|1o5d]], [[1qfk|1qfk]], [[1w0y|1w0y]], [[1w2k|1w2k]], [[1w7x|1w7x]], [[1w8b|1w8b]], [[1ygc|1ygc]], [[1z6j|1z6j]]</td></tr> | ||
<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Coagulation_factor_VIIa Coagulation factor VIIa], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.21 3.4.21.21] </span></td></tr> | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Coagulation_factor_VIIa Coagulation factor VIIa], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.21 3.4.21.21] </span></td></tr> | ||
<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2bz6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2bz6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2bz6 RCSB], [http://www.ebi.ac.uk/pdbsum/2bz6 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=2bz6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2bz6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2bz6 RCSB], [http://www.ebi.ac.uk/pdbsum/2bz6 PDBsum]</span></td></tr> | ||
<table> | </table> | ||
== Disease == | == Disease == | ||
[[http://www.uniprot.org/uniprot/FA7_HUMAN FA7_HUMAN]] Defects in F7 are the cause of factor VII deficiency (FA7D) [MIM:[http://omim.org/entry/227500 227500]]. A hemorrhagic disease with variable presentation. The clinical picture can be very severe, with the early occurrence of intracerebral hemorrhages or repeated hemarthroses, or, in contrast, moderate with cutaneous-mucosal hemorrhages (epistaxis, menorrhagia) or hemorrhages provoked by a surgical intervention. Finally, numerous subjects are completely asymptomatic despite very low factor VII levels.<ref>PMID:8043443</ref> <ref>PMID:2070047</ref> <ref>PMID:1634227</ref> <ref>PMID:8364544</ref> <ref>PMID:8204879</ref> <ref>PMID:7981691</ref> <ref>PMID:7974346</ref> <ref>PMID:8652821</ref> <ref>PMID:8844208</ref> <ref>PMID:8940045</ref> <ref>PMID:8883260</ref> <ref>PMID:9414278</ref> <ref>PMID:9576180</ref> <ref>PMID:9452082</ref> <ref>PMID:11091194</ref> <ref>PMID:11129332</ref> <ref>PMID:10862079</ref> <ref>PMID:12472587</ref> <ref>PMID:14717781</ref> <ref>PMID:19751712</ref> <ref>PMID:18976247</ref> <ref>PMID:19432927</ref> <ref>PMID:21206266</ref> <ref>PMID:21372693</ref> | [[http://www.uniprot.org/uniprot/FA7_HUMAN FA7_HUMAN]] Defects in F7 are the cause of factor VII deficiency (FA7D) [MIM:[http://omim.org/entry/227500 227500]]. A hemorrhagic disease with variable presentation. The clinical picture can be very severe, with the early occurrence of intracerebral hemorrhages or repeated hemarthroses, or, in contrast, moderate with cutaneous-mucosal hemorrhages (epistaxis, menorrhagia) or hemorrhages provoked by a surgical intervention. Finally, numerous subjects are completely asymptomatic despite very low factor VII levels.<ref>PMID:8043443</ref> <ref>PMID:2070047</ref> <ref>PMID:1634227</ref> <ref>PMID:8364544</ref> <ref>PMID:8204879</ref> <ref>PMID:7981691</ref> <ref>PMID:7974346</ref> <ref>PMID:8652821</ref> <ref>PMID:8844208</ref> <ref>PMID:8940045</ref> <ref>PMID:8883260</ref> <ref>PMID:9414278</ref> <ref>PMID:9576180</ref> <ref>PMID:9452082</ref> <ref>PMID:11091194</ref> <ref>PMID:11129332</ref> <ref>PMID:10862079</ref> <ref>PMID:12472587</ref> <ref>PMID:14717781</ref> <ref>PMID:19751712</ref> <ref>PMID:18976247</ref> <ref>PMID:19432927</ref> <ref>PMID:21206266</ref> <ref>PMID:21372693</ref> | ||
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[[Category: Coagulation factor VIIa]] | [[Category: Coagulation factor VIIa]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Ackermann, J | [[Category: Ackermann, J]] | ||
[[Category: Alig, L | [[Category: Alig, L]] | ||
[[Category: Banner, D W | [[Category: Banner, D W]] | ||
[[Category: Boehm, H J | [[Category: Boehm, H J]] | ||
[[Category: Groebke-Zbinden, K | [[Category: Groebke-Zbinden, K]] | ||
[[Category: Hilpert, K | [[Category: Hilpert, K]] | ||
[[Category: Kuehne, H | [[Category: Kuehne, H]] | ||
[[Category: Lave, T | [[Category: Lave, T]] | ||
[[Category: Obst-Sander, U | [[Category: Obst-Sander, U]] | ||
[[Category: Riederer, M A | [[Category: Riederer, M A]] | ||
[[Category: Stahl, M | [[Category: Stahl, M]] | ||
[[Category: Tschopp, T B | [[Category: Tschopp, T B]] | ||
[[Category: Weber, L | [[Category: Weber, L]] | ||
[[Category: Wessel, H P | [[Category: Wessel, H P]] | ||
[[Category: Coagulation]] | [[Category: Coagulation]] | ||
[[Category: Enzyme complex]] | [[Category: Enzyme complex]] | ||
[[Category: Hydrolase]] | [[Category: Hydrolase]] | ||
[[Category: Serine protease]] | [[Category: Serine protease]] | ||
