Involved in several processes, including mitochondrial respirasome assembly; phototaxis; and visual behavior. Predicted to be located in mitochondrial inner membrane. Predicted to be active in mitochondrion. Is expressed in adult head. Used to study Leigh disease and mitochondrial complex IV deficiency nuclear type 1. Human ortholog(s) of this gene implicated in Charcot-Marie-Tooth disease type 4K; Leigh disease; and mitochondrial complex IV deficiency nuclear type 1. Orthologous to human SURF1 (SURF1 cytochrome c oxidase assembly factor).
FB Description
Surfeit 1 (Surf1) encodes a nuclear gene encoding a protein that might be involved in the assembly of the mitochondrial respiratory chain Cytochrome Oxidase (COX or Complex IV). Mutations in its human ortholog Surf1 are related to Leigh Syndrome with COX deficiency (LScox). Surf1 RNAi knockdown produces many of the hallmark signs of LScox, albeit with a more complex biochemical profile.