6PCF

Human Coa6: W59C mutant protein

6PCF の概要

• エントリーDOI: 10.2210/pdb6pcf/pdb
• 関連するPDBエントリー: 6PCE
• 分子名称: Cytochrome c oxidase assembly factor 6 homolog (2 entities in total)
• 機能のキーワード: coa6 copper cytochrome c oxidase mitochondria structure, metal binding protein
• 由来する生物種: Homo sapiens (Human)
• タンパク質・核酸の鎖数: 4
• 化学式量合計: 31571.92

構造登録者

Maher, M.J.,Maghool, S. (登録日: 2019-06-17, 公開日: 2019-10-02, 最終更新日: 2024-11-20)

主引用文献

Maghool, S.,Cooray, N.D.G.,Stroud, D.A.,Aragao, D.,Ryan, M.T.,Maher, M.J.
Structural and functional characterization of the mitochondrial complex IV assembly factor Coa6.
Life Sci Alliance, 2:-, 2019

PubMed Abstract: Assembly factors play key roles in the biogenesis of many multi-subunit protein complexes regulating their stability, activity, and the incorporation of essential cofactors. The human assembly factor Coa6 participates in the biogenesis of the Cu site in complex IV (cytochrome oxidase, COX). Patients with mutations in Coa6 suffer from mitochondrial disease due to complex IV deficiency. Here, we present the crystal structures of human Coa6 and the pathogenic Coa6-mutant protein. These structures show that Coa6 has a 3-helical bundle structure, with the first 2 helices tethered by disulfide bonds, one of which likely provides the copper-binding site. Disulfide-mediated oligomerization of the Coa6 protein provides a structural explanation for the loss-of-function mutation.

PubMed: 31515291
DOI: 10.26508/lsa.201900458

実験手法

X-RAY DIFFRACTION (2.2 Å)