6bq1

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<SX load='6bq1' size='340' side='right' viewer='molstar' caption='[[6bq1]], [[Resolution|resolution]] 3.60&Aring;' scene=''>
<SX load='6bq1' size='340' side='right' viewer='molstar' caption='[[6bq1]], [[Resolution|resolution]] 3.60&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6bq1]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6BQ1 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6BQ1 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6bq1]] is a 6 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=6BQ1 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6BQ1 FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=E4S:5-{2-amino-1-[4-(morpholin-4-yl)phenyl]-1H-benzimidazol-6-yl}-N-(2-fluorophenyl)-2-methoxypyridine-3-sulfonamide'>E4S</scene></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.6&#8491;</td></tr>
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<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=UNK:UNKNOWN'>UNK</scene></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=E4S:5-{2-amino-1-[4-(morpholin-4-yl)phenyl]-1H-benzimidazol-6-yl}-N-(2-fluorophenyl)-2-methoxypyridine-3-sulfonamide'>E4S</scene></td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PI4KA, PIK4, PIK4CA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), TTC7B, TTC7L1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), FAM126A, DRCTNNB1A ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=6bq1 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bq1 OCA], [https://pdbe.org/6bq1 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6bq1 RCSB], [https://www.ebi.ac.uk/pdbsum/6bq1 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6bq1 ProSAT]</span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/1-phosphatidylinositol_4-kinase 1-phosphatidylinositol 4-kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.1.67 2.7.1.67] </span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6bq1 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bq1 OCA], [http://pdbe.org/6bq1 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6bq1 RCSB], [http://www.ebi.ac.uk/pdbsum/6bq1 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6bq1 ProSAT]</span></td></tr>
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</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/HYCCI_HUMAN HYCCI_HUMAN]] Hypomyelination - congenital cataract. The disease is caused by mutations affecting the gene represented in this entry.
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[https://www.uniprot.org/uniprot/PI4KA_HUMAN PI4KA_HUMAN] Bilateral perisylvian polymicrogyria;Autosomal recessive spastic paraplegia type 84;Combined immunodeficiency-enteropathy spectrum. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry.
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/HYCCI_HUMAN HYCCI_HUMAN]] May have a role in the beta-catenin/Lef signaling pathway. May have a role in the process of myelination of the central and peripheral nervous system.<ref>PMID:16951682</ref>
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[https://www.uniprot.org/uniprot/PI4KA_HUMAN PI4KA_HUMAN] Acts on phosphatidylinositol (PtdIns) in the first committed step in the production of the second messenger inositol-1,4,5,-trisphosphate.<ref>PMID:10101268</ref> <ref>PMID:23229899</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Plasma membrane (PM) phosphoinositides play essential roles in cell physiology, serving as both markers of membrane identity and signaling molecules central to the cell's interaction with its environment. The first step in PM phosphoinositide synthesis is the conversion of phosphatidylinositol (PI) to PI4P, the precursor of PI(4,5)P2 and PI(3,4,5)P3 This conversion is catalyzed by the PI4KIIIalpha complex, comprising a lipid kinase, PI4KIIIalpha, and two regulatory subunits, TTC7 and FAM126. We here report the structure of this complex at 3.6-A resolution, determined by cryo-electron microscopy. The proteins form an obligate approximately 700-kDa superassembly with a broad surface suitable for membrane interaction, toward which the kinase active sites are oriented. The structural complexity of the assembly highlights PI4P synthesis as a major regulatory junction in PM phosphoinositide homeostasis. Our studies provide a framework for further exploring the mechanisms underlying PM phosphoinositide regulation.
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Architecture of the human PI4KIIIalpha lipid kinase complex.,Lees JA, Zhang Y, Oh MS, Schauder CM, Yu X, Baskin JM, Dobbs K, Notarangelo LD, De Camilli P, Walz T, Reinisch KM Proc Natl Acad Sci U S A. 2017 Dec 11. pii: 1718471115. doi:, 10.1073/pnas.1718471115. PMID:29229838<ref>PMID:29229838</ref>
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==See Also==
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*[[Phosphatidylinositol 4-kinase|Phosphatidylinositol 4-kinase]]
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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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<div class="pdbe-citations 6bq1" style="background-color:#fffaf0;"></div>
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== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</SX>
</SX>
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[[Category: 1-phosphatidylinositol 4-kinase]]
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[[Category: Homo sapiens]]
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[[Category: Human]]
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[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Baskin, J]]
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[[Category: Baskin J]]
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[[Category: Camilli, P D]]
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[[Category: Camilli PD]]
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[[Category: Dobbs, K]]
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[[Category: Dobbs K]]
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[[Category: Lees, J A]]
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[[Category: Lees JA]]
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[[Category: Notarangelo, L D]]
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[[Category: Notarangelo LD]]
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[[Category: Oh, M]]
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[[Category: Oh M]]
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[[Category: Reinisch, K M]]
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[[Category: Reinisch KM]]
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[[Category: Schauder, C M]]
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[[Category: Schauder CM]]
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[[Category: Walz, T]]
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[[Category: Walz T]]
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[[Category: Yu, X]]
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[[Category: Yu X]]
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[[Category: Zhang, Y]]
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[[Category: Zhang Y]]
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[[Category: Kinase]]
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[[Category: Phosphoinositide synthesis]]
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[[Category: Transferase-signaling protein complex]]
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Current revision

Human PI4KIIIa lipid kinase complex

6bq1, resolution 3.60Å

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