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PDBsum entry 2qz4
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Contents |
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* Residue conservation analysis
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PDB id:
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Hydrolase
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Title:
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Human paraplegin, aaa domain in complex with adp
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Structure:
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Paraplegin. Chain: a. Fragment: aaa domain: residues 305-565. Synonym: spastic paraplegia protein 7. Engineered: yes
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Source:
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Homo sapiens. Human. Organism_taxid: 9606. Tissue: brain. Gene: spg7. Expressed in: escherichia coli. Expression_system_taxid: 562.
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Resolution:
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2.22Å
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R-factor:
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0.212
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R-free:
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0.262
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Authors:
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T.Karlberg,L.Lehtio,C.H.Arrowsmith,H.Berglund,R.D.Busam,R.Collins, L.G.Dahlgren,A.Edwards,S.Flodin,A.Flores,S.Graslund,M.Hammarstrom, M.D.Herman,I.Johansson,A.Kallas,T.Kotenyova,M.Moche,M.E.Nilsson, P.Nordlund,T.Nyman,J.Persson,C.Sagemark,M.Sundstrom,A.G.Thorsell, L.Tresauges,S.Van Den Berg,J.Weigelt,M.Welin,L.Holmberg-Schiavone, Structural Genomics Consortium (Sgc)
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Key ref:
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T.Karlberg
et al.
(2009).
Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7.
Plos One,
4,
e6975.
PubMed id:
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Date:
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16-Aug-07
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Release date:
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11-Sep-07
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PROCHECK
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Headers
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References
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Q9UQ90
(SPG7_HUMAN) -
Paraplegin from Homo sapiens
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Seq:
Struc:
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795 a.a.
223 a.a.*
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Key: |
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PfamA domain |
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Secondary structure |
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CATH domain |
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*
PDB and UniProt seqs differ
at 2 residue positions (black
crosses)
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Plos One
4:e6975
(2009)
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PubMed id:
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Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7.
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T.Karlberg,
S.van den Berg,
M.Hammarström,
J.Sagemark,
I.Johansson,
L.Holmberg-Schiavone,
H.Schüler.
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ABSTRACT
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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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Literature references that cite this PDB file's key reference
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PubMed id
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Reference
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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.
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BMC Neurosci,
11,
55.
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The most recent references are shown first.
Citation data come partly from CiteXplore and partly
from an automated harvesting procedure. Note that this is likely to be
only a partial list as not all journals are covered by
either method. However, we are continually building up the citation data
so more and more references will be included with time.
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Links
