Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

摘要

The Mia40/Erv1 disulfide relay system forms a structural disulfide bond in Ccs1, an unconventional substrate of this system. Thereby it promotes import of Ccs1 into mitochondria and controls its cellular distribution. Thus this system is unexpectedly able to form single disulfide bonds in complex multidomain proteins.