2l7t

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[[Image:2l7t.png|left|200px]]
 
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{{STRUCTURE_2l7t| PDB=2l7t | SCENE= }}
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==Solution structure of the MFS-bound Sans CEN2 peptide==
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<StructureSection load='2l7t' size='340' side='right'caption='[[2l7t]]' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2l7t]] is a 1 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=2L7T OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2L7T 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">Solution NMR</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=2l7t FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2l7t OCA], [https://pdbe.org/2l7t PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2l7t RCSB], [https://www.ebi.ac.uk/pdbsum/2l7t PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2l7t ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/USH1G_HUMAN USH1G_HUMAN] Defects in USH1G are the cause of Usher syndrome type 1G (USH1G) [MIM:[https://omim.org/entry/606943 606943]. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa and sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness.<ref>PMID:21709241</ref> <ref>PMID:12588794</ref> <ref>PMID:20142502</ref> <ref>PMID:16283141</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/USH1G_HUMAN USH1G_HUMAN] Required for normal development and maintenance of cochlear hair cell bundles. Anchoring/scaffolding protein that is a part of the functional network formed by USH1C, USH1G, CDH23 and MYO7A that mediates mechanotransduction in cochlear hair cells. Required for normal hearing.<ref>PMID:21709241</ref> <ref>PMID:12588794</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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The unconventional myosin VIIa (MYO7A) is one of the five proteins that form a network of complexes involved in formation of stereocilia. Defects in these proteins cause syndromic deaf-blindness in humans [Usher syndrome I (USH1)]. Many disease-causing mutations occur in myosin tail homology 4-protein 4.1, ezrin, radixin, moesin (MyTH4-FERM) domains in the myosin tail that binds to another USH1 protein, Sans. We report the crystal structure of MYO7A MyTH4-FERM domains in complex with the central domain (CEN) of Sans at 2.8 angstrom resolution. The MyTH4 and FERM domains form an integral structural and functional supramodule binding to two highly conserved segments (CEN1 and 2) of Sans. The MyTH4-FERM/CEN complex structure provides mechanistic explanations for known deafness-causing mutations in MYO7A MyTH4-FERM. The structure will also facilitate mechanistic and functional studies of MyTH4-FERM domains in other myosins.
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===Solution structure of the MFS-bound Sans CEN2 peptide===
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Structure of MyTH4-FERM domains in myosin VIIa tail bound to cargo.,Wu L, Pan L, Wei Z, Zhang M Science. 2011 Feb 11;331(6018):757-60. PMID:21311020<ref>PMID:21311020</ref>
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{{ABSTRACT_PUBMED_21311020}}
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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==About this Structure==
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<div class="pdbe-citations 2l7t" style="background-color:#fffaf0;"></div>
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[[2l7t]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2L7T OCA].
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== References ==
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<references/>
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==Reference==
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__TOC__
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<ref group="xtra">PMID:021311020</ref><references group="xtra"/>
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</StructureSection>
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[[Category: Pan, L.]]
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[[Category: Homo sapiens]]
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[[Category: Wei, Z.]]
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[[Category: Large Structures]]
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[[Category: Wu, L.]]
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[[Category: Pan L]]
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[[Category: Zhang, M.]]
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[[Category: Wei Z]]
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[[Category: Protein peptide]]
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[[Category: Wu L]]
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[[Category: Unknown function]]
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[[Category: Zhang M]]

Current revision

Solution structure of the MFS-bound Sans CEN2 peptide

PDB ID 2l7t

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