3u7d

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[[Image:3u7d.png|left|200px]]
 
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{{STRUCTURE_3u7d| PDB=3u7d | SCENE= }}
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==Crystal structure of the KRIT1/CCM1 FERM domain in complex with the heart of glass (HEG1) cytoplasmic tail==
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<StructureSection load='3u7d' size='340' side='right'caption='[[3u7d]], [[Resolution|resolution]] 2.49&Aring;' scene=''>
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===Crystal structure of the KRIT1/CCM1 FERM domain in complex with the heart of glass (HEG1) cytoplasmic tail===
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3u7d]] is a 4 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=3U7D OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3U7D FirstGlance]. <br>
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{{ABSTRACT_PUBMED_23007647}}
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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]] 2.49&#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=3u7d FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3u7d OCA], [https://pdbe.org/3u7d PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3u7d RCSB], [https://www.ebi.ac.uk/pdbsum/3u7d PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3u7d ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[3u7d]] is a 4 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=3U7D OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/KRIT1_HUMAN KRIT1_HUMAN] Hereditary cerebral cavernous malformation. Cerebral cavernous malformations 1 (CCM1) [MIM:[https://omim.org/entry/116860 116860]: A congenital vascular anomaly of the central nervous system that can result in hemorrhagic stroke, seizures, recurrent headaches, and focal neurologic deficits. The lesions are characterized by grossly enlarged blood vessels consisting of a single layer of endothelium and without any intervening neural tissue, ranging in diameter from a few millimeters to several centimeters. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12172908</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/KRIT1_HUMAN KRIT1_HUMAN] Component of the CCM signaling pathway which is a crucial regulator of heart and vessel formation and integrity (By similarity). Negative regulator of angiogenesis. Inhibits endothelial proliferation, apoptosis, migration, lumen formation and sprouting angiogenesis in primary endothelial cells. Promotes AKT phosphorylation in a NOTCH-dependent and independent manner, and inhibits EKR1/2 phosphorylation indirectly through activation of the DELTA-NOTCH cascade. Acts in concert with CDH5 to establish and maintain correct endothelial cell polarity and vascular lumen and these effects are mediated by recruitment and activation of the Par polarity complex and RAP1B. Required for the localization of phosphorylated PRKCZ, PARD3, TIAM1 and RAP1B to the cell junction, and cell junction stabilization. Plays a role in integrin signaling via its interaction with ITGB1BP1; this prevents the interaction between ITGB1 and ITGB1BP1. Plays an important role in the maintenance of the intracellular reactive oxygen species (ROS) homeostasis to prevent oxidative cellular damage. Regulates the homeostasis of intracellular ROS through an antioxidant pathway involving FOXO1 and SOD2. Facilitates the down-regulation of cyclin-D1 (CCND1) levels required for cell transition from proliferative growth to quiescence by preventing the accumulation of intracellular ROS through the modulation of FOXO1 and SOD2 levels.<ref>PMID:20332120</ref> <ref>PMID:20668652</ref> <ref>PMID:20616044</ref> <ref>PMID:21633110</ref> [REFERENCE:17]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Assembly, Dynamics and Evolution of Cell-Cell and Cell-Matrix Adhesions CELLMAT]]
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[[Category: Large Structures]]
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[[Category: Gingras, A R.]]
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[[Category: Gingras AR]]
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[[Category: Ginsberg, M H.]]
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[[Category: Ginsberg MH]]
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[[Category: Liu, J J.]]
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[[Category: Liu JJ]]
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[[Category: Assembly]]
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[[Category: Cellmat]]
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[[Category: Dynamics and evolution of cell-cell and cell-matrix adhesion]]
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[[Category: Ferm domain]]
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[[Category: Membrane protein cytoplasmic tail]]
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[[Category: Protein binding]]
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[[Category: Psi-biology]]
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[[Category: Rap1 effector]]
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Current revision

Crystal structure of the KRIT1/CCM1 FERM domain in complex with the heart of glass (HEG1) cytoplasmic tail

PDB ID 3u7d

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