3U2M

Crystal structure of human ALR mutant C142/145S

3U2M の概要

• エントリーDOI: 10.2210/pdb3u2m/pdb
• 関連するPDBエントリー: 3O55 3U2L
• 分子名称: FAD-linked sulfhydryl oxidase ALR, FLAVIN-ADENINE DINUCLEOTIDE (3 entities in total)
• 機能のキーワード: flavoprotein, alr, sulfhydryl oxidase, flavin, fad
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Isoform 1: Mitochondrion intermembrane space. Isoform 2: Cytoplasm: P55789
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 14467.72

構造登録者

Banci, L.,Bertini, I.,Calderone, V.,Cefaro, C.,Ciofi-Baffoni, S.,Gallo, A. (登録日: 2011-10-04, 公開日: 2012-06-06, 最終更新日: 2024-11-20)

主引用文献

Banci, L.,Bertini, I.,Calderone, V.,Cefaro, C.,Ciofi-Baffoni, S.,Gallo, A.,Tokatlidis, K.
An electron-transfer path through an extended disulfide relay system: the case of the redox protein ALR.
J.Am.Chem.Soc., 134:1442-1445, 2012

PubMed Abstract: The oxidative folding mechanism in the intermembrane space of human mitochondria underpins a disulfide relay system consisting of the import receptor Mia40 and the homodimeric FAD-dependent thiol oxidase ALR. The flavoprotein ALR receives two electrons per subunit from Mia40, which are then donated through one-electron reactions to two cytochrome c molecules, thus mediating a switch from two-electron to one-electron transfer. We dissect here the mechanism of the electron flux within ALR, characterizing at the atomic level the ALR intermediates that allow electrons to rapidly flow to cytochrome c. The intermediate critical for the electron-transfer process implies the formation of a specific inter-subunit disulfide which exclusively allows electron flow from Mia40 to FAD. This finding allows us to present a complete model for the electron-transfer pathway in ALR.

PubMed: 22224850
DOI: 10.1021/ja209881f

実験手法

X-RAY DIFFRACTION (2 Å)