SURF1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Invitrogen
Rabbit Polyclonal Antibody
Manufacturer: Invitrogen PA549828
The antibody detects endogenous levels of total SURF-1 protein.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.
|Synthesized peptide derived from internal of human SURF1.|
|Antigen affinity chromatography|
|Human, Mouse, Rat|
|PBS with 50% glycerol and 0.02% sodium azide; pH 7.4|
|Q15526, P09925, Q9QXU2|
|Surfeit locus protein 1, SURF1, SURF-1,|
|20930, 64463, 6834|
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