PROSITE documentation PDOC01040
SURF1 family profile


The surfeit locus 1 gene (SURF1 or surf-1) encodes for a conserved protein of about 300 amino-acid residues that seems to be involved in the biogenesis of cytochrome c oxidase [1]. Vertebrate SURF1 is evolutionary related to yeast protein SHY1 and homologous proteins have been found in several relatively recent prokaryotes. All known eukaryotic SURF1 sequences are extended on the N-terminal end as compared to the bacterial species. This extension contains a typical mitochondrial targeting pre-sequence. In Paracoccus denitrificans, the SURF1 homologue is found in the quinol oxidase operon, suggesting that SURF1 is associated with a primitive quinol oxidase which belongs to the same superfamily as cytochrome oxidase [2].

Proteins of the SURF1 family contain at least four highly conserved regions that should be essential for SURF1 function and there seems to be two transmembrane regions in these proteins, one in the N-terminal, the other in the C-terminal [2].

The profile we developed covers the two transmembrane domains and the region in between.


This profile replaces a pattern which sensitivity was inadequate.

Last update:

January 2003 / Pattern removed, profile added and text revised.


Technical section

PROSITE method (with tools and information) covered by this documentation:

SURF1, PS50895; SURF1 family profile  (MATRIX)


1AuthorsZhu Z. Yao J. Johns T. Fu K. De Bie I. MacMillan C. Cuthbert A.P. Newbold R.F. Wang J. Chevrette M. Brown G.K. Brown R.M. Shoubridge E.A.
TitleSURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome.
SourceNat. Genet. 20:337-343(1998).
PubMed ID9843204

2AuthorsPoyau A. Buchet K. Godinot C.
TitleSequence conservation from human to prokaryotes of Surf1, a protein involved in cytochrome c oxidase assembly, deficient in Leigh syndrome.
SourceFEBS Lett. 462:416-420(1999).
PubMed ID10622737

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