3e9l

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{{STRUCTURE_3e9l| PDB=3e9l | SCENE= }}
 
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===Crystal Structure of Human Prp8, Residues 1755-2016===
 
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{{ABSTRACT_PUBMED_18843295}}
 
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==Disease==
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==Crystal Structure of Human Prp8, Residues 1755-2016==
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[[http://www.uniprot.org/uniprot/PRP8_HUMAN PRP8_HUMAN]] Defects in PRPF8 are the cause of retinitis pigmentosa type 13 (RP13) [MIM:[http://omim.org/entry/600059 600059]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP13 inheritance is autosomal dominant.<ref>PMID:17317632</ref><ref>PMID:11468273</ref>[:]<ref>PMID:11910553</ref><ref>PMID:12714658</ref>
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<StructureSection load='3e9l' size='340' side='right'caption='[[3e9l]], [[Resolution|resolution]] 1.95&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3e9l]] is a 1 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=3E9L OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3E9L FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/PRP8_HUMAN PRP8_HUMAN]] Central component of the spliceosome, which may play a role in aligning the pre-mRNA 5'- and 3'-exons for ligation. Interacts with U5 snRNA, and with pre-mRNA 5'-splice sites in B spliceosomes and 3'-splice sites in C spliceosomes.
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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]] 1.95&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene></td></tr>
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==About this Structure==
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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=3e9l FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3e9l OCA], [https://pdbe.org/3e9l PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3e9l RCSB], [https://www.ebi.ac.uk/pdbsum/3e9l PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3e9l ProSAT]</span></td></tr>
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[[3e9l]] is a 1 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=3E9L OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PRP8_HUMAN PRP8_HUMAN] Defects in PRPF8 are the cause of retinitis pigmentosa type 13 (RP13) [MIM:[https://omim.org/entry/600059 600059]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP13 inheritance is autosomal dominant.<ref>PMID:17317632</ref> <ref>PMID:11468273</ref> [:]<ref>PMID:11910553</ref> <ref>PMID:12714658</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/PRP8_HUMAN PRP8_HUMAN] Central component of the spliceosome, which may play a role in aligning the pre-mRNA 5'- and 3'-exons for ligation. Interacts with U5 snRNA, and with pre-mRNA 5'-splice sites in B spliceosomes and 3'-splice sites in C spliceosomes.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/e9/3e9l_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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=3e9l ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[Pre-mRNA-splicing factor|Pre-mRNA-splicing factor]]
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*[[Pre-mRNA splicing factors 3D structures|Pre-mRNA splicing factors 3D structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:018843295</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Pena, V.]]
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[[Category: Large Structures]]
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[[Category: Rozov, A.]]
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[[Category: Pena V]]
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[[Category: Wahl, M C.]]
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[[Category: Rozov A]]
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[[Category: Disease mutation]]
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[[Category: Wahl MC]]
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[[Category: Mrna processing]]
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[[Category: Mrna splicing]]
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[[Category: Nucleotidyl transfer]]
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[[Category: Nucleus]]
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[[Category: Phosphoprotein]]
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[[Category: Retinitis pigmentosa]]
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[[Category: Rna binding protein]]
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[[Category: Rna-binding]]
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[[Category: Sensory transduction]]
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[[Category: Spliceosome]]
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[[Category: Splicing]]
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[[Category: Vision]]
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Current revision

Crystal Structure of Human Prp8, Residues 1755-2016

PDB ID 3e9l

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