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Anti-Surf1 Rabbit pAb (100 μl)
| Reactivity: | M,R |
| Applications: | WB |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Full Name: | Surf1 rabbit polyclonal antibody |
Gene Name: | Surfeit locus protein 1 |
Synonyms: | Surf1, Surf-1, 0610010F23Rik, Ab1-205, CMT4K |
Immunogen: | Recombinant protein corresponding to Mouse Surf1 |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. | 35 kDa |
Observed MW. | 30 kDa |
Uniprot ID: | P09925 |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
WB | Mouse, Rat | 1: 500-1: 1000 | heart, kidney, stomach |
Background
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.
Images
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Western blot analysis of SURF1 (GB111526) at dilution of 1: 500 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
