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2qz4

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Human paraplegin, AAA domain in complex with ADP

Structural highlights

2qz4 is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:	X-ray diffraction, Resolution 2.22Å
Ligands:
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

SPG7_HUMAN Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7) [MIM: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.^[1] ^[2] ^[3] ^[4] 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.

Function

SPG7_HUMAN Putative ATP-dependent zinc metalloprotease.

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

Publication Abstract from PubMed

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.

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^[5]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

References

↑ Casari G, De Fusco M, Ciarmatori S, Zeviani M, Mora M, Fernandez P, De Michele G, Filla A, Cocozza S, Marconi R, Durr A, Fontaine B, Ballabio A. Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease. Cell. 1998 Jun 12;93(6):973-83. PMID:9635427
↑ Elleuch N, Depienne C, Benomar A, Hernandez AM, Ferrer X, Fontaine B, Grid D, Tallaksen CM, Zemmouri R, Stevanin G, Durr A, Brice A. Mutation analysis of the paraplegin gene (SPG7) in patients with hereditary spastic paraplegia. Neurology. 2006 Mar 14;66(5):654-9. PMID:16534102 doi:10.1212/01.wnl.0000201185.91110.15
↑ Warnecke T, Duning T, Schwan A, Lohmann H, Epplen JT, Young P. A novel form of autosomal recessive hereditary spastic paraplegia caused by a new SPG7 mutation. Neurology. 2007 Jul 24;69(4):368-75. PMID:17646629 doi:69/4/368
↑ Bonn F, Pantakani K, Shoukier M, Langer T, Mannan AU. Functional evaluation of paraplegin mutations by a yeast complementation assay. Hum Mutat. 2010 May;31(5):617-21. doi: 10.1002/humu.21226. PMID:20186691 doi:10.1002/humu.21226
↑ Karlberg T, van den Berg S, Hammarstrom M, Sagemark J, Johansson I, Holmberg-Schiavone L, Schuler H. Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7. PLoS One. 2009 Oct 20;4(10):e6975. PMID:19841671 doi:10.1371/journal.pone.0006975

1 Structural highlights
2 Disease
3 Function
4 Evolutionary Conservation
5 Publication Abstract from PubMed
6 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/2qz4"

@@ Line 3: / Line 3: @@
 <StructureSection load='2qz4' size='340' side='right'caption='[[2qz4]], [[Resolution|resolution]] 2.22&Aring;' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[2qz4]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. 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>
+<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>
-</td></tr><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>
+</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>
-<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">SPG7 ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
+<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>
 <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>
 </table>
 == Disease ==
-[[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.
+[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.
 == Function ==
-[[https://www.uniprot.org/uniprot/SPG7_HUMAN SPG7_HUMAN]] Putative ATP-dependent zinc metalloprotease.
+[https://www.uniprot.org/uniprot/SPG7_HUMAN SPG7_HUMAN] Putative ATP-dependent zinc metalloprotease.
 == Evolutionary Conservation ==
 [[Image:Consurf_key_small.gif|200px|right]]
@@ Line 35: / Line 35: @@
 __TOC__
 </StructureSection>
-[[Category: Human]]
+[[Category: Homo sapiens]]
 [[Category: Large Structures]]
-[[Category: Arrowsmith, C H]]
+[[Category: Arrowsmith CH]]
-[[Category: Berg, S Van Den]]
+[[Category: Berglund H]]
-[[Category: Berglund, H]]
+[[Category: Busam RD]]
-[[Category: Busam, R D]]
+[[Category: Collins R]]
-[[Category: Collins, R]]
+[[Category: Dahlgren LG]]
-[[Category: Dahlgren, L G]]
+[[Category: Edwards A]]
-[[Category: Edwards, A]]
+[[Category: Flodin S]]
-[[Category: Flodin, S]]
+[[Category: Flores A]]
-[[Category: Flores, A]]
+[[Category: Graslund S]]
-[[Category: Graslund, S]]
+[[Category: Hammarstrom M]]
-[[Category: Hammarstrom, M]]
+[[Category: Herman MD]]
-[[Category: Herman, M D]]
+[[Category: Holmberg-Schiavone L]]
-[[Category: Holmberg-Schiavone, L]]
+[[Category: Johansson I]]
-[[Category: Johansson, I]]
+[[Category: Kallas A]]
-[[Category: Kallas, A]]
+[[Category: Karlberg T]]
-[[Category: Karlberg, T]]
+[[Category: Kotenyova T]]
-[[Category: Kotenyova, T]]
+[[Category: Lehtio L]]
-[[Category: Lehtio, L]]
+[[Category: Moche M]]
-[[Category: Moche, M]]
+[[Category: Nilsson ME]]
-[[Category: Nilsson, M E]]
+[[Category: Nordlund P]]
-[[Category: Nordlund, P]]
+[[Category: Nyman T]]
-[[Category: Nyman, T]]
+[[Category: Persson J]]
-[[Category: Persson, J]]
+[[Category: Sagemark C]]
-[[Category: Structural genomic]]
+[[Category: Sundstrom M]]
-[[Category: Sagemark, C]]
+[[Category: Thorsell AG]]
-[[Category: Sundstrom, M]]
+[[Category: Tresauges L]]
-[[Category: Thorsell, A G]]
+[[Category: Van Den Berg S]]
-[[Category: Tresauges, L]]
+[[Category: Weigelt J]]
-[[Category: Weigelt, J]]
+[[Category: Welin M]]
-[[Category: Welin, M]]
-[[Category: Aaa+]]
-[[Category: Adp]]
-[[Category: Atp-binding]]
-[[Category: Hydrolase]]
-[[Category: Nucleotide-binding]]
-[[Category: Protease]]
-[[Category: Sgc]]
-[[Category: Spg7]]

2qz4

From Proteopedia

Current revision

Human paraplegin, AAA domain in complex with ADP

Structural highlights

Disease

Function

Evolutionary Conservation

Publication Abstract from PubMed

References

Contents

Proteopedia Page Contributors and Editors (what is this?)

Views

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