PMID- 17298220
OWN - NLM
STAT- MEDLINE
DCOM- 20070529
LR  - 20220531
IS  - 0862-8408 (Print)
IS  - 0862-8408 (Linking)
VI  - 55 Suppl 2
DP  - 2006
TI  - Biogenesis of eukaryotic cytochrome c oxidase.
PG  - S27-41
AB  - Eukaryotic cytochrome c oxidase (CcO), the terminal component of the 
      mitochondrial electron transport chain is a heterooligomeric complex that belongs 
      to the superfamily of heme-copper containing terminal oxidases. The enzyme, 
      composed of both mitochondrially and nuclear encoded subunits, is embedded in the 
      inner mitochondrial membrane, where it catalyzes the transfer of electrons form 
      reduced cytochrome c to dioxygen, coupling this reaction with vectorial proton 
      pumping across the inner membrane. Due to the complexity of the enzyme, the 
      biogenesis of CcO involves a multiplicity of steps, carried out by a number of 
      highly specific gene products. These include mainly proteins that mediate the 
      delivery and insertion of copper ions, synthesis and incorporation of heme 
      moieties and membrane-insertion and topogenesis of constituent protein subunits. 
      Isolated CcO deficiency represents one of the most frequently recognized causes 
      of respiratory chain defects in humans, associated with severe, often fatal 
      clinical phenotype. Here we review recent advancements in the understanding of 
      this intricate process, with a focus on mammalian enzyme.
FAU - Stiburek, L
AU  - Stiburek L
AD  - Department of Pediatrics, First Faculty of Medicine, Charles University, Prague, 
      Czech Republic.
FAU - Hansikova, H
AU  - Hansikova H
FAU - Tesarova, M
AU  - Tesarova M
FAU - Cerna, L
AU  - Cerna L
FAU - Zeman, J
AU  - Zeman J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Czech Republic
TA  - Physiol Res
JT  - Physiological research
JID - 9112413
RN  - 0 (Protein Subunits)
RN  - 42VZT0U6YR (Heme)
RN  - 789U1901C5 (Copper)
RN  - EC 1.9.3.1 (Electron Transport Complex IV)
SB  - IM
MH  - Animals
MH  - Copper/metabolism
MH  - Electron Transport
MH  - Electron Transport Complex IV/*chemistry/*metabolism
MH  - Heme/metabolism
MH  - Humans
MH  - Mitochondrial Membranes/enzymology/metabolism
MH  - Models, Biological
MH  - Protein Subunits/metabolism
RF  - 117
EDAT- 2007/02/15 09:00
MHDA- 2007/05/30 09:00
CRDT- 2007/02/15 09:00
PHST- 2007/02/15 09:00 [pubmed]
PHST- 2007/05/30 09:00 [medline]
PHST- 2007/02/15 09:00 [entrez]
AID - 10.33549/physiolres.930000.55.S2.27 [doi]
PST - ppublish
SO  - Physiol Res. 2006;55 Suppl 2:S27-41. doi: 10.33549/physiolres.930000.55.S2.27.