SURF1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
Manufacturer: Proteintech Group Inc 153791AP
DescriptionThe 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.
|PBS with 50% glycerol and 0.02% sodium azide; pH 7.3|
|P09925, Q15526, Q9QXU2|
|SURF 1, SURF1, surfeit 1, Surfeit locus protein 1|
|20930, 64463, 6834|
|Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Immunoprecipitation, Western Blot|
|SURF1 Fusion Protein Ag3959|
|Antigen Affinity Chromatography|
|Human, Mouse, Rat|
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