2k9u: Difference between revisions

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 ==Solution NMR structure of the Filamin-migfilin complex==
-<StructureSection load='2k9u' size='340' side='right'caption='[[2k9u]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
+<StructureSection load='2k9u' size='340' side='right'caption='[[2k9u]]' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[2k9u]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2K9U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2K9U FirstGlance]. <br>
+<table><tr><td colspan='2'>[[2k9u]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2K9U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2K9U FirstGlance]. <br>
-</td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2k9u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2k9u OCA], [https://pdbe.org/2k9u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2k9u RCSB], [https://www.ebi.ac.uk/pdbsum/2k9u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2k9u ProSAT]</span></td></tr>
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2k9u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2k9u OCA], [https://pdbe.org/2k9u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2k9u RCSB], [https://www.ebi.ac.uk/pdbsum/2k9u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2k9u ProSAT]</span></td></tr>
 </table>
+== Disease ==
+[https://www.uniprot.org/uniprot/FLNC_HUMAN FLNC_HUMAN] Defects in FLNC are the cause of myopathy myofibrillar type 5 (MFM5) [MIM:[https://omim.org/entry/609524 609524]. A neuromuscular disorder, usually with an adult onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations, and clinical features of a limb-girdle myopathy.<ref>PMID:15929027</ref>   Defects in FLNC are the cause of myopathy distal type 4 (MPD4) [MIM:[https://omim.org/entry/614065 614065]. MPD4 is a slowly progressive muscular disorder characterized by distal muscle weakness and atrophy affecting the upper and lower limbs. Onset occurs around the third to fourth decades of life, and patients remain ambulatory even after long disease duration. Muscle biopsy shows non-specific changes with no evidence of rods, necrosis, or inflammation.<ref>PMID:21620354</ref>
 == Function ==
-[[https://www.uniprot.org/uniprot/FBLI1_HUMAN FBLI1_HUMAN]] Serves as an anchoring site for cell-ECM adhesion proteins and filamin-containing actin filaments. Is implicated in cell shape modulation (spreading) and motility. May participate in the regulation of filamin-mediated cross-linking and stabilization of actin filaments. May also regulate the assembly of filamin-containing signaling complexes that control actin assembly. Promotes dissociation of FLNA from ITGB3 and ITGB7. Promotes activation of integrins and regulates integrin-mediated cell-cell adhesion.<ref>PMID:12679033</ref> <ref>PMID:12496242</ref> <ref>PMID:18829455</ref> <ref>PMID:19074766</ref>
+[https://www.uniprot.org/uniprot/FLNC_HUMAN FLNC_HUMAN] Muscle-specific filamin, which plays a central role in muscle cells, probably by functioning as a large actin-cross-linking protein. May be involved in reorganizing the actin cytoskeleton in response to signaling events, and may also display structural functions at the Z lines in muscle cells. Critical for normal myogenesis and for maintaining the structural integrity of the muscle fibers.
 <div style="background-color:#fffaf0;">
 == Publication Abstract from PubMed ==
@@ Line 25: / Line 28: @@
 __TOC__
 </StructureSection>
-[[Category: Human]]
+[[Category: Homo sapiens]]
 [[Category: Large Structures]]
-[[Category: Ithychanda, S N]]
+[[Category: Ithychanda SN]]
-[[Category: Qin, J]]
+[[Category: Qin J]]
-[[Category: Cell adhesion]]
-[[Category: Cell junction]]
-[[Category: Cell shape]]
-[[Category: Cytoskeletal complex]]
-[[Category: Cytoskeleton]]
-[[Category: Lim domain]]
-[[Category: Metal-binding]]
-[[Category: Structural protein]]