The domain within your query sequence starts at position 73 and ends at position 290; the E-value for the SURF1 domain shown below is 5.9e-64.


PFAM accession number:PF02104
Interpro abstract (IPR002994):

This entry includes SURF1 and Shy1 proteins. The surfeit locus 1 gene (SURF1 or surf-1) encodes a conserved protein of about 300 amino-acid residues that seems to be involved in the biogenesis of cytochrome c oxidase [ (PUBMED:9843204) ]. Vertebrate SURF1 is evolutionary related to yeast protein Shy1, which is a mitochondrial inner membrane protein required for assembly of cytochrome c oxidase [ (PUBMED:11782424) ]. There seems to be two transmembrane regions in these proteins, one in the N-terminal, the other in the C-terminal.

Defects in SURF1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterised by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency [ (PUBMED:9843204) (PUBMED:10746561) (PUBMED:10647889) ].

GO component:membrane (GO:0016020)

This is a PFAM domain. For full annotation and more information, please see the PFAM entry SURF1