PDBsum entry 2qz4

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Hydrolase PDB id
Protein chain
223 a.a. *
Waters ×55
* Residue conservation analysis
PDB id:
Name: Hydrolase
Title: Human paraplegin, aaa domain in complex with adp
Structure: Paraplegin. Chain: a. Fragment: aaa domain: residues 305-565. Synonym: spastic paraplegia protein 7. Engineered: yes
Source: Homo sapiens. Human. Organism_taxid: 9606. Tissue: brain. Gene: spg7. Expressed in: escherichia coli. Expression_system_taxid: 562.
2.22Å     R-factor:   0.212     R-free:   0.262
Authors: T.Karlberg,L.Lehtio,C.H.Arrowsmith,H.Berglund,R.D.Busam,R.Co L.G.Dahlgren,A.Edwards,S.Flodin,A.Flores,S.Graslund,M.Hamma M.D.Herman,I.Johansson,A.Kallas,T.Kotenyova,M.Moche,M.E.Nil P.Nordlund,T.Nyman,J.Persson,C.Sagemark,M.Sundstrom,A.G.Tho L.Tresauges,S.Van Den Berg,J.Weigelt,M.Welin,L.Holmberg-Sch Structural Genomics Consortium (Sgc)
Key ref: T.Karlberg et al. (2009). Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7. PLoS One, 4, e6975. PubMed id: 19841671
16-Aug-07     Release date:   11-Sep-07    
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Protein chain
Pfam   ArchSchema ?
Q9UQ90  (SPG7_HUMAN) -  Paraplegin
795 a.a.
223 a.a.*
Key:    PfamA domain  PfamB domain  Secondary structure  CATH domain
* PDB and UniProt seqs differ at 2 residue positions (black crosses)

 Gene Ontology (GO) functional annotation 
  GO annot!
  Biochemical function     ATP binding     1 term  


PLoS One 4:e6975 (2009)
PubMed id: 19841671  
Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7.
T.Karlberg, S.van den Berg, M.Hammarström, J.Sagemark, I.Johansson, L.Holmberg-Schiavone, H.Schüler.
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.

Literature references that cite this PDB file's key reference

  PubMed id Reference
20426821 T.Sacco, E.Boda, E.Hoxha, R.Pizzo, C.Cagnoli, A.Brusco, and F.Tempia (2010).
Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease.
  BMC Neurosci, 11, 55.  
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