MitoProteome Database

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MT000748

UniProt Annotations

Entry Information

Gene Name	COX17 cytochrome c oxidase copper chaperone
Protein Entry	COX17_HUMAN
UniProt ID	Q14061
Species	Human

Comments

Comment type	Description
Function	Copper chaperone for cytochrome c oxidase (COX). Binds two copper ions and deliver them to the Cu(A) site of COX (By similarity). {ECO:0000250}.
Similarity	Belongs to the COX17 family. {ECO:0000305}.
Subcellular Location	Mitochondrion intermembrane space {ECO:0000250}.
Subunit	Interacts with COA1. Interacts with the chaperone CHCHD4; this is important for correct folding and the formation of disulfide bonds that stabilize the structure. {ECO:0000269\|PubMed:21059946, ECO:0000269\|PubMed:22356826}.
Tissue Specificity	Ubiquitous. {ECO:0000269\|PubMed:10982038}.