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Mitochondrial copper handling. Pic2 imports copper from the intermembrane space (IMS) to the lumen, where it is bound to a ligand (CuL). This copper is thought to be exported by an unidentified transporter from the matrix into the IMS, where it is acquired by Cox17 and transferred to Sco1/2 or Cox11 for insertion into COX subunits, or acquired by Ccs for insertion into Sod1. Coa6 plays a critical role in COX metalation and assembly through currently unknown mechanisms.
