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Literature summary for 7.1.1.9 extracted from

  • Bundschuh, F.A.; Hannappel, A.; Anderka, O.; Ludwig, B.
    Surf1, associated with Leigh syndrome in humans, is a heme-binding protein in bacterial oxidase biogenesis (2009), J. Biol. Chem., 284, 25735-25741.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 7.1.1.9

Activating Compound

Activating Compound Comment Organism Structure
Surf1 biogenesis of mitochondrial cytochrome c oxidase relies on a large number of assembly factors, among them the transmembrane protein Surf1 Homo sapiens

Application

Application Comment Organism
medicine mutations in various mitochondrial enzymes can result in Leigh syndrome, among them cytochrome c oxidase Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Metals/Ions

Metals/Ions Comment Organism Structure
copper
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ferrocytochrome c + O2 + H+ Homo sapiens
-
 ferricytochrome c + H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ferrocytochrome c + O2 + H+
-
 Homo sapiens ferricytochrome c + H2O
-
 ?

Subunits

Subunits Comment Organism
oligomer mitochondrial COX consists of up to 13 subunits Homo sapiens

Synonyms

Synonyms Comment Organism
COX
-
 Homo sapiens
cytochrome c oxidase
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
heme a
-
 Homo sapiens

Expression

Organism Comment Expression
Homo sapiens Leigh syndrome most frequent trigger is deficiency of cytochrome c oxidase caused by mutations in the SURF1 gene down