2QZ4

Human paraplegin, AAA domain in complex with ADP

Summary for 2QZ4

• Entry DOI: 10.2210/pdb2qz4/pdb
• Descriptor: Paraplegin, ADENOSINE-5'-DIPHOSPHATE (3 entities in total)
• Functional Keywords: aaa+, spg7, protease, adp, structural genomics, structural genomics consortium, sgc, atp-binding, nucleotide-binding, hydrolase
• Biological source: Homo sapiens (human)
• Total number of polymer chains: 1
• Total formula weight: 28979.09

Authors

Karlberg, T.,Lehtio, L.,Arrowsmith, C.H.,Berglund, H.,Busam, R.D.,Collins, R.,Dahlgren, L.G.,Edwards, A.,Flodin, S.,Flores, A.,Graslund, S.,Hammarstrom, M.,Herman, M.D.,Johansson, I.,Kallas, A.,Kotenyova, T.,Moche, M.,Nilsson, M.E.,Nordlund, P.,Nyman, T.,Persson, J.,Sagemark, C.,Sundstrom, M.,Thorsell, A.G.,Tresauges, L.,Van Den Berg, S.,Weigelt, J.,Welin, M.,Holmberg-Schiavone, L.,Structural Genomics Consortium (SGC) (deposition date: 2007-08-16, release date: 2007-09-11, Last modification date: 2023-08-30)

Primary citation

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, 4:e6975-e6975, 2009

PubMed Abstract: 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.

PubMed: 19841671
DOI: 10.1371/journal.pone.0006975

Experimental method

X-RAY DIFFRACTION (2.22 Å)