This gene encodes a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency.
Categories
Primary Antibodies
Clonality
polyclonal
Description
May play a role in mitochondrial respiratory chain complex IV assembly (PubMed:24027061). Probably involved in the biogenesis of the COX complex (PubMed:9843204).
Host
Rabbit
Immunogen
surfeit 1
Isotype
IgG
Molecular Weight
33 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Synonyms
SURF-1
Uniprot
Q15526
Gene Id
6834
Research Area
Metabolism
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)