4wtx: Difference between revisions

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-'''Unreleased structure'''
+==Crystal structure of the fourth FnIII domain of integrin beta4==
+<StructureSection load='4wtx' size='340' side='right' caption='[[4wtx]], [[Resolution|resolution]] 1.50&Aring;' scene=''>
-The entry 4wtx is ON HOLD  until Paper Publication
+== Structural highlights ==
+<table><tr><td colspan='2'>[[4wtx]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4WTX OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4WTX FirstGlance]. <br>
-Authors: Alonso-Garcia, N., Urien, H., Buey, R.M., de Pereda, J.M.
+</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=4wtx FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4wtx OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4wtx RCSB], [http://www.ebi.ac.uk/pdbsum/4wtx PDBsum]</span></td></tr>
+</table>
-Description: Crystal structure of the fourth FnIII domain of integrin beta4
+== Disease ==
-[[Category: Unreleased Structures]]
+[[http://www.uniprot.org/uniprot/ITB4_HUMAN ITB4_HUMAN]] Defects in ITGB4 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:[http://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:[http://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>
-[[Category: De Pereda, J.M]]
+== Function ==
+[[http://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>
+== References ==
+<references/>
+__TOC__
+</StructureSection>
+[[Category: Alonso-Garcia, N]]
+[[Category: Buey, R M]]
+[[Category: Pereda, J M.de]]
 [[Category: Urien, H]]
-[[Category: Buey, R.M]]
+[[Category: Cell adhesion]]
-[[Category: Alonso-Garcia, N]]
+[[Category: Fibronectin type iii]]
+[[Category: Integrin]]
+[[Category: Receptor]]