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PDBsum entry 2qz4
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References listed in PDB file
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Key reference
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Title
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Crystal structure of the atpase domain of the human aaa+ protein paraplegin/spg7.
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Authors
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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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Ref.
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Plos One, 2009,
4,
e6975.
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PubMed id
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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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