1PE0

Crystal structure of the K130R mutant of human DJ-1

1PE0 の概要

• エントリーDOI: 10.2210/pdb1pe0/pdb
• 関連するPDBエントリー: 1pdv 1pdw
• 分子名称: DJ-1 (2 entities in total)
• 機能のキーワード: dj-1, protein binding
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Cytoplasm: Q99497
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 42032.44

構造登録者

Tao, X.,Tong, L. (登録日: 2003-05-20, 公開日: 2003-06-24, 最終更新日: 2024-02-14)

主引用文献

Tao, X.,Tong, L.
Crystal Structure of Human DJ-1, a Protein Associated with Early Onset Parkinson's Disease.
J.Biol.Chem., 278:31372-31379, 2003

PubMed Abstract: We report the crystal structure at 1.8-A resolution of human DJ-1, which has been linked to early onset Parkinson's disease. The monomer of DJ-1 contains the alpha/beta-fold that is conserved among members of the DJ-1/ThiJ/PfpI superfamily. However, the structure also contains an extra helix at the C terminus, which mediates a novel mode of dimerization for the DJ-1 proteins. A putative active site has been identified near the dimer interface, and the residues Cys-106, His-126, and Glu-18 may play important roles in the catalysis by this protein. Studies with the disease-causing L166P mutant suggest that the mutation has disrupted the C-terminal region and the dimerization of the protein. The DJ-1 proteins may function only as dimers. The Lys to Arg mutation at residue 130, the site of sumoylation of DJ-1, has minimal impact on the structure of the protein.

PubMed: 12761214
DOI: 10.1074/jbc.M304221200

実験手法

X-RAY DIFFRACTION (1.7 Å)