[HTML][HTML] Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies

D Pacheu-Grau, B Bareth, J Dudek, L Juris, FN Vögtle… - Cell metabolism, 2015 - cell.com
Three mitochondria-encoded subunits form the catalytic core of cytochrome c oxidase, the
terminal enzyme of the respiratory chain. COX1 and COX2 contain heme and copper redox …

[HTML][HTML] Folding studies of Cox17 reveal an important interplay of cysteine oxidation and copper binding

F Arnesano, E Balatri, L Banci, I Bertini, DR Winge - Structure, 2005 - cell.com
Cox17 is a key mitochondrial copper chaperone involved in the assembly of cytochrome c
oxidase (COX). The NMR solution structure of the oxidized apoCox17 isoform consists of a …

Metal-binding mechanism of Cox17, a copper chaperone for cytochrome c oxidase

P Palumaa, L Kangur, A Voronova… - Biochemical …, 2004 - portlandpress.com
Cox17, a copper chaperone for cytochrome c oxidase, is an essential and highly conserved
protein. The structure and mechanism of functioning of Cox17 are unknown, and even its …

[HTML][HTML] HIGD2A is required for assembly of the COX3 module of human mitochondrial complex IV

DH Hock, B Reljic, CS Ang, L Muellner-Wong… - Molecular & Cellular …, 2020 - ASBMB
Assembly factors play a critical role in the biogenesis of mitochondrial respiratory chain
complexes I-IV where they assist in the membrane insertion of subunits, attachment of co …

[HTML][HTML] Yeast Cox17 solution structure and copper (I) binding

C Abajian, LA Yatsunyk, BE Ramirez… - Journal of Biological …, 2004 - ASBMB
Cox17 is a 69-residue cysteine-rich, copper-binding protein that has been implicated in the
delivery of copper to the Cu A and Cu B centers of cytochrome c oxidase via the copper …

Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7

K Mohanraj, M Wasilewski, C Benincá… - EMBO molecular …, 2019 - embopress.org
Nuclear and mitochondrial genome mutations lead to various mitochondrial diseases, many
of which affect the mitochondrial respiratory chain. The proteome of the intermembrane …

A yeast suppressor screen links Coa4 to the mitochondrial copper delivery pathway for cytochrome c oxidase

AB Swaminathan, S Soma, AC Vicary, M Zulkifli… - Genetics, 2022 - academic.oup.com
Cytochrome c oxidase (CcO) is a multimeric copper-containing enzyme of the mitochondrial
respiratory chain that powers cellular energy production. The two core subunits of …

Redox regulation of SCO protein function: controlling copper at a mitochondrial crossroad

SC Leary - Antioxidants & redox signaling, 2010 - liebertpub.com
Reversible changes in the redox state of cysteine residues represent an important
mechanism with which to regulate protein function. In mitochondria, such redox reactions …

[HTML][HTML] A structural-dynamical characterization of human Cox17

L Banci, I Bertini, S Ciofi-Baffoni, A Janicka… - Journal of Biological …, 2008 - ASBMB
Human Cox17 is a key mitochondrial copper chaperone responsible for supplying copper
ions, through the assistance of Sco1, Sco2, and Cox11, to cytochrome c oxidase, the …

Mitochondrial copper metabolism and delivery to cytochrome c oxidase

D Horn, A Barrientos - IUBMB life, 2008 - Wiley Online Library
Metals are essential elements of all living organisms. Among them, copper is required for a
multiplicity of functions including mitochondrial oxidative phosphorylation and protection …