1tzn

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{{Large structure}}
 
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{{STRUCTURE_1tzn| PDB=1tzn | SCENE= }}
 
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===Crystal Structure of the Anthrax Toxin Protective Antigen Heptameric Prepore bound to the VWA domain of CMG2, an anthrax toxin receptor===
 
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{{ABSTRACT_PUBMED_15326297}}
 
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==Disease==
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==Crystal Structure of the Anthrax Toxin Protective Antigen Heptameric Prepore bound to the VWA domain of CMG2, an anthrax toxin receptor==
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[[http://www.uniprot.org/uniprot/ANTR2_HUMAN ANTR2_HUMAN]] Defects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH) [MIM:[http://omim.org/entry/236490 236490]]. This autosomal recessive syndrome is similar to JHF, but has an earlier onset and a more severe course. Symptoms appear at birth or within the first months of life, with painful, swollen joint contractures, osteopenia, osteoporosis and livid red hyperpigmentation over bony prominences. Patients develop multiple subcutaneous skin tumors and gingival hypertrophy. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to death within the first 2 years of life. Surviving children may suffer from severely reduced mobility due to joint contractures.<ref>PMID:12973667</ref><ref>PMID:14508707</ref> Defects in ANTXR2 are the cause of juvenile hyaline fibromatosis (JHF) [MIM:[http://omim.org/entry/228600 228600]]. JHF is an autosomal recessive syndrome that is similar to ISH but takes a milder course. It is characterized by hyaline deposition in the dermis, multiple subcutaneous skin tumors and gingival hypertrophy, followed by progressive joint contractions, osteopenia and osteoporosis that may lead to a severe limitation of mobility.<ref>PMID:12973667</ref><ref>PMID:14508707</ref>
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<StructureSection load='1tzn' size='340' side='right'caption='[[1tzn]], [[Resolution|resolution]] 4.30&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[1tzn]] is a 28 chain structure with sequence from [https://en.wikipedia.org/wiki/Bacillus_anthracis_str._A2012 Bacillus anthracis str. A2012] and [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1TZN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1TZN FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/PAG_BACAN PAG_BACAN]] One of the three proteins composing the anthrax toxin, the agent which infects many mammalian species and that may cause death. PA binds to a receptor (ATR) in sensitive eukaryotic cells, thereby facilitating the translocation of the enzymatic toxin components, edema factor and lethal factor, across the target cell membrane. PA associated with LF causes death when injected, PA associated with EF produces edema. PA induces immunity to infection with anthrax. [[http://www.uniprot.org/uniprot/ANTR2_HUMAN ANTR2_HUMAN]] Necessary for cellular interactions with laminin and the extracellular matrix.<ref>PMID:11683410</ref><ref>PMID:12973667</ref>
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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]] 4.3&#8491;</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=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></td></tr>
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==About this Structure==
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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=1tzn FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1tzn OCA], [https://pdbe.org/1tzn PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1tzn RCSB], [https://www.ebi.ac.uk/pdbsum/1tzn PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1tzn ProSAT]</span></td></tr>
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[[1tzn]] is a 28 chain structure with sequence from [http://en.wikipedia.org/wiki/Bacillus_anthracis_str._a2012 Bacillus anthracis str. a2012]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1TZN OCA].
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</table>
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== Function ==
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==See Also==
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[https://www.uniprot.org/uniprot/PAG_BACAN PAG_BACAN] One of the three proteins composing the anthrax toxin, the agent which infects many mammalian species and that may cause death. PA binds to a receptor (ATR) in sensitive eukaryotic cells, thereby facilitating the translocation of the enzymatic toxin components, edema factor and lethal factor, across the target cell membrane. PA associated with LF causes death when injected, PA associated with EF produces edema. PA induces immunity to infection with anthrax.
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*[[Anthrax protective antigen|Anthrax protective antigen]]
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== Evolutionary Conservation ==
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*[[Jmol/Visualizing large molecules|Jmol/Visualizing large molecules]]
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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==Reference==
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<jmolCheckbox>
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<ref group="xtra">PMID:015326297</ref><references group="xtra"/><references/>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/tz/1tzn_consurf.spt"</scriptWhenChecked>
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[[Category: Bacillus anthracis str. a2012]]
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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[[Category: Collier, R J.]]
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<text>to colour the structure by Evolutionary Conservation</text>
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[[Category: Lacy, D B.]]
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</jmolCheckbox>
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[[Category: Melnyk, R A.]]
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1tzn ConSurf].
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[[Category: Wigelsworth, D J.]]
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<div style="clear:both"></div>
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[[Category: Heptamer]]
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__TOC__
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[[Category: Toxin receptor-toxin complex]]
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</StructureSection>
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[[Category: Bacillus anthracis str. A2012]]
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Collier RJ]]
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[[Category: Lacy DB]]
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[[Category: Melnyk RA]]
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[[Category: Wigelsworth DJ]]

Current revision

Crystal Structure of the Anthrax Toxin Protective Antigen Heptameric Prepore bound to the VWA domain of CMG2, an anthrax toxin receptor

PDB ID 1tzn

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