Molecular weight (DA)
30kD
Immunogen
The antiserum was produced against synthesized peptide derived from human SURF1. AA range:171-220
Specificity
SURF-1 Polyclonal Antibody detects endogenous levels of SURF-1 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/20000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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],
Function
disease: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.,
Protein name
Surfeit locus protein 1
Other name
SURF1; SURF-1; Surfeit locus protein 1
Human protein sequence Database
Q15526
Mouse protein sequence database
P09925
Rat protein sequence database
Q9QXU2
Cellular localization
Mitochondrion inner membrane ; Multi-pass membrane protein .
Tissue expression
Colon,Kidney,Skin,Stomach,
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