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SURF1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech Non-distribution product as customer accommodation.

Rabbit Polyclonal Antibody

Manufacturer:  Proteintech Group IncSupplier Diversity Partner 153791AP

Catalog No. 50-173-0189

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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.


0.17 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
P09925, Q15526, Q9QXU2
SURF 1, SURF1, surfeit 1, Surfeit locus protein 1
150 μL
20930, 64463, 6834
Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Immunoprecipitation, Western Blot
SURF1 Fusion Protein Ag3959
Antigen Affinity Chromatography
Human, Mouse, Rat
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