PMID- 17008312
OWN - NLM
STAT- MEDLINE
DCOM- 20070130
LR  - 20210209
IS  - 0021-9258 (Print)
IS  - 0021-9258 (Linking)
VI  - 281
IP  - 48
DP  - 2006 Dec 1
TI  - Mitochondrial matrix copper complex used in metallation of cytochrome oxidase and 
      superoxide dismutase.
PG  - 36552-9
AB  - A mitochondrial matrix copper ligand (CuL) complex, conserved in mammalian cells, 
      is the likely source of copper for assembly of cytochrome c oxidase (CcO) and 
      superoxide dismutase 1 (Sod1) within the intermembrane space (IMS) in yeast. 
      Targeting the copper-binding proteins human Sod1 and Crs5 to the mitochondrial 
      matrix results in growth impairment on non-fermentable medium caused by decreased 
      levels of CcO. This effect is reversed by copper supplementation. Matrix-targeted 
      Crs5 diminished Sod1 protein within the IMS and impaired activity of an inner 
      membrane tethered human Sod1. Copper binding by the matrix-targeted proteins 
      attenuates levels of the CuL complex without affecting total mitochondrial 
      copper. These data suggest that attenuation of the matrix CuL complex via 
      heterologous competitors limits available copper for metallation of CcO and Sod1 
      within the IMS. The ligand also exists in the cytoplasm in an apparent metal-free 
      state.
FAU - Cobine, Paul A
AU  - Cobine PA
AD  - University of Utah Health Sciences Center, Departments of Medicine and 
      Biochemistry, Salt Lake City, Utah 84132, USA.
FAU - Pierrel, Fabien
AU  - Pierrel F
FAU - Bestwick, Megan L
AU  - Bestwick ML
FAU - Winge, Dennis R
AU  - Winge DR
LA  - eng
GR  - ES03817/ES/NIEHS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20060928
PL  - United States
TA  - J Biol Chem
JT  - The Journal of biological chemistry
JID - 2985121R
RN  - 0 (CRS5 protein, S cerevisiae)
RN  - 0 (Ligands)
RN  - 0 (Molecular Chaperones)
RN  - 0 (SOD1 protein, human)
RN  - 0 (Saccharomyces cerevisiae Proteins)
RN  - 789U1901C5 (Copper)
RN  - 9038-94-2 (Metallothionein)
RN  - EC 1.15.1.1 (Superoxide Dismutase)
RN  - EC 1.15.1.1 (Superoxide Dismutase-1)
RN  - EC 1.9.3.1 (Electron Transport Complex IV)
RN  - PDC6A3C0OX (Glycerol)
SB  - IM
MH  - Biological Transport
MH  - Copper/chemistry/*metabolism
MH  - Cytoplasm/metabolism
MH  - Electron Transport Complex IV/*metabolism
MH  - Genetic Complementation Test
MH  - Glycerol/chemistry
MH  - Humans
MH  - Ligands
MH  - Metallothionein/metabolism
MH  - Mitochondria/enzymology/*metabolism
MH  - Molecular Chaperones
MH  - Oxidative Stress
MH  - Phenotype
MH  - Saccharomyces cerevisiae Proteins/metabolism
MH  - Superoxide Dismutase/chemistry/*metabolism
MH  - Superoxide Dismutase-1
EDAT- 2006/09/30 09:00
MHDA- 2007/01/31 09:00
CRDT- 2006/09/30 09:00
PHST- 2006/09/30 09:00 [pubmed]
PHST- 2007/01/31 09:00 [medline]
PHST- 2006/09/30 09:00 [entrez]
AID - S0021-9258(20)71999-X [pii]
AID - 10.1074/jbc.M606839200 [doi]
PST - ppublish
SO  - J Biol Chem. 2006 Dec 1;281(48):36552-9. doi: 10.1074/jbc.M606839200. Epub 2006 
      Sep 28.