3fso: Difference between revisions

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[[Image:3fso.png|left|200px]]


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==Crystal structure of the Calx-beta domain of integrin beta4, calcium soak==
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<StructureSection load='3fso' size='340' side='right'caption='[[3fso]], [[Resolution|resolution]] 1.41&Aring;' scene=''>
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== Structural highlights ==
or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
<table><tr><td colspan='2'>[[3fso]] is a 2 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=3FSO OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3FSO FirstGlance]. <br>
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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]] 1.405&#8491;</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=3fso FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3fso OCA], [https://pdbe.org/3fso PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3fso RCSB], [https://www.ebi.ac.uk/pdbsum/3fso PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3fso ProSAT]</span></td></tr>
{{STRUCTURE_3fso| PDB=3fso |  SCENE= }}
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ITB4_HUMAN ITB4_HUMAN] Defects in ITGB4 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:[https://omim.org/entry/226730 226730]; also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is an autosomal recessive, frequently lethal, epidermolysis bullosa with variable involvement of skin, nails, mucosa, and with variable effects on the digestive system. It is characterized by mucocutaneous fragility, aplasia cutis congenita, and gastrointestinal atresia, which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa.<ref>PMID:9792864</ref> <ref>PMID:9422533</ref> <ref>PMID:9546354</ref> <ref>PMID:9892956</ref> <ref>PMID:10873890</ref> <ref>PMID:11251584</ref> <ref>PMID:11328943</ref>  Defects in ITGB4 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:[https://omim.org/entry/226650 226650]. GABEB is a non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities.<ref>PMID:10792571</ref>
== Function ==
[https://www.uniprot.org/uniprot/ITB4_HUMAN ITB4_HUMAN] Integrin alpha-6/beta-4 is a receptor for laminin. Plays a critical structural role in the hemidesmosome of epithelial cells. Is required for the regulation of keratinocyte polarity and motility.<ref>PMID:12482924</ref> <ref>PMID:19403692</ref>
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/fs/3fso_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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=3fso ConSurf].
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== Publication Abstract from PubMed ==
The integrin alpha6beta4 is a receptor for laminins and provides stable adhesion of epithelial cells to the basement membranes. In addition, alpha6beta4 is important for keratinocyte migration during wound healing and favours the invasion of carcinomas into surrounding tissue. The cytoplasmic domain of the beta4 subunit is responsible for most of the intracellular interactions of the integrin; it contains four fibronectin type III domains and a Calx-beta motif. The crystal structure of the Calx-beta domain of beta4 was determined to 1.48 A resolution. The structure does not contain cations and biophysical data support the supposition that the Calx-beta domain of beta4 does not bind calcium. Comparison of the Calx-beta domain of beta4 with the calcium-binding domains of Na(+)/Ca(2+)-exchanger 1 reveals that in beta4 Arg1003 occupies a position equivalent to that of the calcium ions in the Na(+)/Ca(2+)-exchanger. By combining mutagenesis and thermally induced unfolding, it is shown that Arg1003 contributes to the stability of the Calx-beta domain. The structure of the Calx-beta domain is discussed in the context of the function and intracellular interactions of the integrin beta4 subunit and a putative functional site is proposed.


===Crystal structure of the Calx-beta domain of integrin beta4, calcium soak===
Structure of the Calx-beta domain of the integrin beta4 subunit: insights into function and cation-independent stability.,Alonso-Garcia N, Ingles-Prieto A, Sonnenberg A, de Pereda JM Acta Crystallogr D Biol Crystallogr. 2009 Aug;65(Pt 8):858-71. Epub 2009, Jul 17. PMID:19622870<ref>PMID:19622870</ref>


From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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<div class="pdbe-citations 3fso" style="background-color:#fffaf0;"></div>


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==See Also==
The line below this paragraph, {{ABSTRACT_PUBMED_19622870}}, adds the Publication Abstract to the page
*[[Integrin 3D structures|Integrin 3D structures]]
(as it appears on PubMed at http://www.pubmed.gov), where 19622870 is the PubMed ID number.
== References ==
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<references/>
{{ABSTRACT_PUBMED_19622870}}
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</StructureSection>
==Disease==
Known disease associated with this structure: Epidermolysis bullosa of hands and feet OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=147557 147557]], Epidermolysis bullosa, junctional, non-Herlitz type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=147557 147557]], Epidermolysis bullosa, junctional, with pyloric atresia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=147557 147557]]
 
==About this Structure==
3FSO is a 2 chains structure of sequences 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=3FSO OCA].
 
==Reference==
<ref group="xtra">PMID:19622870</ref><references group="xtra"/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Alonso-Garcia, N.]]
[[Category: Large Structures]]
[[Category: Ingles-Prieto, A.]]
[[Category: Alonso-Garcia N]]
[[Category: Pereda, J M.de.]]
[[Category: Ingles-Prieto A]]
[[Category: Alternative splicing]]
[[Category: De Pereda JM]]
[[Category: Cell adhesion]]
[[Category: Disease mutation]]
[[Category: Epidermolysis bullosa]]
[[Category: Glycoprotein]]
[[Category: Immunoglobulin fold]]
[[Category: Integrin]]
[[Category: Membrane]]
[[Category: Phosphoprotein]]
[[Category: Polymorphism]]
[[Category: Receptor]]
[[Category: Transmembrane]]
 
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