Synthetic peptide, corresponding to amino acids 150-200 of Human SURF-1.
Konjugation:
Unconjugated
Alternative Synonym:
Surfeit locus protein 1, SURF1, SURF-1
The SURF-1 protein demonstrates a vital role in the assembly of complex IV (CIV or COX) of the mitochondrial respiratory chain. Expressed in the inner mitochondrial membrane, mutations of the SURF-1 gene generally cause cytochrome c oxidase complex IV deficiency. Shortage of complex IV leads to Leigh syndrome, a severe neurological disorder. Leigh syndrome patients are usually subject to rapidly progressive encephalopathy, characterized by necrotic lesions in subcortical brain regions. SURF-1 mutations correlate to high post-implantation embryonic lethality as well as early-onset mortality of post-natal individuals. Considerable deficit in muscle strength and motor performance is also a profound and isolated defect of SURF-1 activity in skeletal muscle and liver. Heart, brain and skeletal muscle morphological abnormalities frequently occur due to SURF-1 mutations.
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Formulierung:
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Application Verdünnung:
IHC: 1:50~1:200
Anwendungsbeschreibung:
SURF-1 (F181) polyclonal antibody detects endogenous levels of SURF-1 protein.
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