2QZ4

Human paraplegin, AAA domain in complex with ADP

2QZ4 の概要

• エントリーDOI: 10.2210/pdb2qz4/pdb
• 分子名称: Paraplegin, ADENOSINE-5'-DIPHOSPHATE (3 entities in total)
• 機能のキーワード: aaa+, spg7, protease, adp, structural genomics, structural genomics consortium, sgc, atp-binding, nucleotide-binding, hydrolase
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 28979.09

構造登録者

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) (登録日: 2007-08-16, 公開日: 2007-09-11, 最終更新日: 2023-08-30)

主引用文献

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

実験手法

X-RAY DIFFRACTION (2.22 Å)