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GeneBio Systems

Anti-SURF1 Antibody Picoband®

Anti-SURF1 Antibody Picoband®

SKU:A03678-2

Regular price ¥94,200 JPY
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Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Monkey,Mouse,Rat

Applications: WB,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: SURF1

Specificity:

Background: Surfeit locus protein 1 (SURF1) is a protein that in humans is encoded by the SURF1 gene. This gene encodes a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a biional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency.

Immunogen: E.coli-derived human SURF1 recombinant protein (Position: S27-R295).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Agostino, A., Invernizzi, F., Tiveron, C., Fagiolari, G., Prelle, A., Lamantea, E., Giavazzi, A., Battaglia, G., Tatangelo, L., Tiranti, V., Zeviani, M. Constitutive knockout of Surf1 is associated with high embryonic lethality, mitochondrial disease and cytochrome c oxidase deficiency in mice. Hum. Molec. Genet. 12: 399-413, 2003. 2. Bohm, M., Pronicka, E., Karczmarewicz, E., Pronicki, M., Piekutowska-Abramczuk, D., Sykut-Cegielska, J., Mierzewska, H., Hansikova, H., Vesela, K., Tesarova, M., Houstkova, H., Houstek, J., Zeman, J. Retrospective, multicentric study of 180 children with cytochrome c oxidase deficiency. Pediat. Res. 59: 21-26, 2006. 3. Colombo, P., Yon, J., Garson, K., Fried, M. Conservation of the organization of five tightly clustered genes over 600 million years of divergent evolution. Proc. Nat. Acad. Sci. 89: 6358-6362, 1992.

Uniprot ID: Q15526

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 33 kDa

Calculated Molecular Weight: 28461 MW

Gene ID: 6834

Protein Name: Surfeit locus protein 1

Gene Full Name: SURF1 cytochrome c oxidase assembly factor

Synonyms: SURF 1; SURF1; surfeit 1; Surfeit locus protein 1

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