protein SCO2 homolog, mitochondrial (SCO2)

Function

• acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit 2 (COX2)

Structure

• belongs to the SCO1/2 family
• contains 1 thioredoxin domain

Compartment

mitochondria

Expression

ubiquitous

Pathology

• defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency

More general terms

• mitochondrial protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=9997
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:9997
• OMIM: https://mirror.omim.org/entry/604272
• OMIM: https://mirror.omim.org/entry/604377
• UniProt: http://www.uniprot.org/uniprot/O43819.html