2qz4

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[[Image:2qz4.png|left|200px]]
 
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{{STRUCTURE_2qz4| PDB=2qz4 | SCENE= }}
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==Human paraplegin, AAA domain in complex with ADP==
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<StructureSection load='2qz4' size='340' side='right'caption='[[2qz4]], [[Resolution|resolution]] 2.22&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2qz4]] 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=2QZ4 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2QZ4 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">X-ray diffraction, [[Resolution|Resolution]] 2.22&#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=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene></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=2qz4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2qz4 OCA], [https://pdbe.org/2qz4 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2qz4 RCSB], [https://www.ebi.ac.uk/pdbsum/2qz4 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2qz4 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/SPG7_HUMAN SPG7_HUMAN] Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7) [MIM:[https://omim.org/entry/607259 607259]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG7 is a complex form. Additional clinical features are cerebellar syndrome, supranuclear palsy, and cognitive impairment, particularly disturbance of attention and executive functions.<ref>PMID:9635427</ref> <ref>PMID:16534102</ref> <ref>PMID:17646629</ref> <ref>PMID:20186691</ref> Note=Defects in SPG7 may cause autosomal recessive osteogenesis imperfecta (OI). Osteogenesis imperfecta defines a group of connective tissue disorders characterized by bone fragility and low bone mass. Clinical features of SPG7-related osteogenesis imperfecta include recurrent fractures, mild bone deformities, delayed tooth eruption, normal hearing and white sclera.
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== Function ==
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[https://www.uniprot.org/uniprot/SPG7_HUMAN SPG7_HUMAN] Putative ATP-dependent zinc metalloprotease.
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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/qz/2qz4_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=2qz4 ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Paraplegin is an m-AAA protease of the mitochondrial inner membrane that is linked to hereditary spastic paraplegias. The gene encodes an FtsH-homology protease domain in tandem with an AAA+ homology ATPase domain. The protein is believed to form a hexamer that uses ATPase-driven conformational changes in its AAA-domain to deliver substrate peptides to its protease domain. We present the crystal structure of the AAA-domain of human paraplegin bound to ADP at 2.2 A. This enables assignment of the roles of specific side chains within the catalytic cycle, and provides the structural basis for understanding the mechanism of disease mutations. ENHANCED VERSION: This article can also be viewed as an enhanced version in which the text of the article is integrated with interactive 3D representations and animated transitions. Please note that a web plugin is required to access this enhanced functionality. Instructions for the installation and use of the web plugin are available in Text S1.
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===Human paraplegin, AAA domain in complex with ADP===
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Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7.,Karlberg T, van den Berg S, Hammarstrom M, Sagemark J, Johansson I, Holmberg-Schiavone L, Schuler H PLoS One. 2009 Oct 20;4(10):e6975. PMID:19841671<ref>PMID:19841671</ref>
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{{ABSTRACT_PUBMED_19841671}}
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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 2qz4" style="background-color:#fffaf0;"></div>
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[[2qz4]] 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=2QZ4 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:019841671</ref><references group="xtra"/>
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Large Structures]]
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[[Category: Berg, S Van Den.]]
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[[Category: Arrowsmith CH]]
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[[Category: Berglund, H.]]
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[[Category: Berglund H]]
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[[Category: Busam, R D.]]
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[[Category: Busam RD]]
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[[Category: Collins, R.]]
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[[Category: Collins R]]
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[[Category: Dahlgren, L G.]]
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[[Category: Dahlgren LG]]
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[[Category: Edwards, A.]]
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[[Category: Edwards A]]
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[[Category: Flodin, S.]]
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[[Category: Flodin S]]
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[[Category: Flores, A.]]
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[[Category: Flores A]]
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[[Category: Graslund, S.]]
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[[Category: Graslund S]]
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[[Category: Hammarstrom, M.]]
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[[Category: Hammarstrom M]]
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[[Category: Herman, M D.]]
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[[Category: Herman MD]]
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[[Category: Holmberg-Schiavone, L.]]
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[[Category: Holmberg-Schiavone L]]
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[[Category: Johansson, I.]]
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[[Category: Johansson I]]
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[[Category: Kallas, A.]]
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[[Category: Kallas A]]
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[[Category: Karlberg, T.]]
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[[Category: Karlberg T]]
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[[Category: Kotenyova, T.]]
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[[Category: Kotenyova T]]
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[[Category: Lehtio, L.]]
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[[Category: Lehtio L]]
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[[Category: Moche, M.]]
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[[Category: Moche M]]
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[[Category: Nilsson, M E.]]
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[[Category: Nilsson ME]]
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[[Category: Nordlund, P.]]
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[[Category: Nordlund P]]
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[[Category: Nyman, T.]]
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[[Category: Nyman T]]
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[[Category: Persson, J.]]
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[[Category: Persson J]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Sagemark C]]
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[[Category: Sagemark, C.]]
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[[Category: Sundstrom M]]
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[[Category: Sundstrom, M.]]
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[[Category: Thorsell AG]]
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[[Category: Thorsell, A G.]]
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[[Category: Tresauges L]]
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[[Category: Tresauges, L.]]
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[[Category: Van Den Berg S]]
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[[Category: Weigelt, J.]]
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[[Category: Weigelt J]]
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[[Category: Welin, M.]]
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[[Category: Welin M]]
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[[Category: Aaa+]]
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[[Category: Adp]]
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[[Category: Atp-binding]]
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[[Category: Hydrolase]]
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[[Category: Nucleotide-binding]]
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[[Category: Protease]]
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[[Category: Sgc]]
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[[Category: Spg7]]
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[[Category: Structural genomic]]
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[[Category: Structural genomics consortium]]
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Current revision

Human paraplegin, AAA domain in complex with ADP

PDB ID 2qz4

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