Product Information
Molecular Weight33331 DaObserved Band:30 KD
Protein NameSurfeit locus protein 1
Human Gene Id6834
Human Swiss Prot NoQ15526
Mouse Gene Id20930
Mouse Swiss Prot NoP09925
Rat Gene Id64463
Rat Swiss Prot NoQ9QXU2
SpecificitySURF-1 Polyclonal Antibody detects endogenous levels of SURF-1 protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NameSURF1; SURF-1; Surfeit locus protein 1
Functiondisease:Defects in SURF1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency.,function:Probably involved in the biogenesis of the COX complex.,similarity:Belongs to the SURF1 family.,
Subcellular LocationMitochondrion inner membrane ; Multi-pass membrane protein .
ExpressionColon,Kidney,Skin,Stomach,
BackgroundSURF1, cytochrome c oxidase assembly factor(SURF1) Homo sapiens 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. [provided by RefSeq, Jul 2008],