PMID- 10622737 OWN - NLM STAT- MEDLINE DCOM- 20000202 LR - 20190621 IS - 0014-5793 (Print) IS - 0014-5793 (Linking) VI - 462 IP - 3 DP - 1999 Dec 3 TI - Sequence conservation from human to prokaryotes of Surf1, a protein involved in cytochrome c oxidase assembly, deficient in Leigh syndrome. PG - 416-20 AB - The human SURF1 gene encoding a protein involved in cytochrome c oxidase (COX) assembly, is mutated in most patients presenting Leigh syndrome associated with COX deficiency. Proteins homologous to the human Surf1 have been identified in nine eukaryotes and six prokaryotes using database alignment tools, structure prediction and/or cDNA sequencing. Their sequence comparison revealed a remarkable Surf1 conservation during evolution and put forward at least four highly conserved domains that should be essential for Surf1 function. 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. FAU - Poyau, A AU - Poyau A AD - Centre de Genetique Moleculaire et Cellulaire, Centre National de la Recherche Scientifique, Universite Claude Bernard Lyon I, Villeurbanne, France. FAU - Buchet, K AU - Buchet K FAU - Godinot, C AU - Godinot C LA - eng SI - GENBANK/AF182952 SI - GENBANK/AF182953 SI - GENBANK/AF182954 PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - FEBS Lett JT - FEBS letters JID - 0155157 RN - 0 (DNA, Complementary) RN - 0 (Membrane Proteins) RN - 0 (Mitochondrial Proteins) RN - 0 (Proteins) RN - 0 (Surf-1 protein) SB - IM MH - Amino Acid Sequence MH - Animals MH - Conserved Sequence MH - DNA, Complementary/metabolism MH - Humans MH - Leigh Disease/*enzymology/*genetics MH - Membrane Proteins MH - Mitochondrial Proteins MH - Molecular Sequence Data MH - Phylogeny MH - Polymerase Chain Reaction MH - Proteins/*genetics MH - Sequence Analysis, DNA MH - Sequence Homology, Amino Acid EDAT- 2000/01/06 00:00 MHDA- 2000/01/06 00:01 CRDT- 2000/01/06 00:00 PHST- 2000/01/06 00:00 [pubmed] PHST- 2000/01/06 00:01 [medline] PHST- 2000/01/06 00:00 [entrez] AID - S0014-5793(99)01571-9 [pii] AID - 10.1016/s0014-5793(99)01571-9 [doi] PST - ppublish SO - FEBS Lett. 1999 Dec 3;462(3):416-20. doi: 10.1016/s0014-5793(99)01571-9.