PMID- 16631971 OWN - NLM STAT- MEDLINE DCOM- 20060927 LR - 20220408 IS - 0006-3002 (Print) IS - 0006-3002 (Linking) VI - 1763 IP - 7 DP - 2006 Jul TI - Copper trafficking to the mitochondrion and assembly of copper metalloenzymes. PG - 759-72 AB - Copper is required within the mitochondrion for the function of two metalloenzymes, cytochrome c oxidase (CcO) and superoxide dismutase (Sod1). Copper metallation of these two enzymes occurs within the mitochondrial intermembrane space and is mediated by metallochaperone proteins. Cox17 is a key copper donor to two accessory proteins, Sco1 and Cox11, to form the two copper centers in the mature CcO complex. Ccs1 is the necessary metallochaperone for the copper metallation of Sod1 in the IMS as well as within the cytoplasm where the bulk of Sod1 resides. Copper ions used in the metallation of CcO and Sod1 appear to be provided by a novel copper pool within the mitochondrial matrix. This review documents copper ion shuttling within the mitochondrion and the proteins that mediate assembly of active CcO and Sod1. FAU - Cobine, Paul A AU - Cobine PA AD - Departments of Medicine and Biochemistry, University of Utah Health Sciences Center, Salt Lake City, UT 84132, USA. FAU - Pierrel, Fabien AU - Pierrel F FAU - Winge, Dennis R AU - Winge DR LA - eng GR - ES 03817/ES/NIEHS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review DEP - 20060331 PL - Netherlands TA - Biochim Biophys Acta JT - Biochimica et biophysica acta JID - 0217513 RN - 789U1901C5 (Copper) RN - EC 1.9.3.1 (Electron Transport Complex IV) SB - IM MH - Biological Transport MH - Copper/*metabolism MH - Electron Transport Complex IV/chemistry/*metabolism MH - Mitochondria/*metabolism MH - Models, Molecular MH - Protein Conformation RF - 94 EDAT- 2006/04/25 09:00 MHDA- 2006/09/28 09:00 CRDT- 2006/04/25 09:00 PHST- 2005/12/21 00:00 [received] PHST- 2006/03/03 00:00 [revised] PHST- 2006/03/05 00:00 [accepted] PHST- 2006/04/25 09:00 [pubmed] PHST- 2006/09/28 09:00 [medline] PHST- 2006/04/25 09:00 [entrez] AID - S0167-4889(06)00053-X [pii] AID - 10.1016/j.bbamcr.2006.03.002 [doi] PST - ppublish SO - Biochim Biophys Acta. 2006 Jul;1763(7):759-72. doi: 10.1016/j.bbamcr.2006.03.002. Epub 2006 Mar 31.