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GeneBio Systems

Anti-COX6B1 Antibody Picoband®

Anti-COX6B1 Antibody Picoband®

SKU:A10285-2

Regular price $618.00 USD
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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,Mouse,Rat

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

Application Details: Western blot, 0.25-0.5 μg/ml, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human

Gene Name: COX6B1

Specificity:

Background: Cytochrome c oxidase subunit 6B1 is an enzyme that in humans is encoded by the COX6B1 gene. Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may be involved in the regulation and assembly of the complex. This nuclear gene encodes subunit VIb. Mutations in this gene are associated with severe infantile encephalomyopathy. Three pseudogenes COX6BP-1, COX6BP-2 and COX6BP-3 have been found on chromosomes 7, 17 and 22q13.1-13.2, respectively.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human COX6B1, identical to the related mouse and rat sequences.

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. Abdulhag, U. N., Soiferman, D., Schueler-Furman, O., Miller, C., Shaag, A., Elpeleg, O., Edvardson, S., Saada, A. Mitochondrial complex IV deficiency, caused by mutated COX6B1, is associated with encephalomyopathy, hydrocephalus and cardiomyopathy. Europ. J. Hum. Genet. 23: 159-164, 2015. 2. Carrero-Valenzuela, R. D., Quan, F., Lightowlers, R., Kennaway, N. G., Litt, M., Forte, M. Human cytochrome c oxidase subunit VIb: characterization and mapping of a multigene family. Gene 102: 229-236, 1991. 3. Massa, V., Fernandez-Vizarra, E., Alshahwan, S., Bakhsh, E., Goffrini, P., Ferrero, I., Mereghetti, P., D'Adamo, P., Gasparini, P., Zeviani, M. Severe infantile encephalomyopathy caused by a mutation in COX6B1, a nucleus-encoded subunit of cytochrome c oxidase. Am. J. Hum. Genet. 82: 1281-1289, 2008.

Uniprot ID: P14854

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: 12 kDa

Calculated Molecular Weight: 41409 MW

Gene ID: 1340

Protein Name: Cytochrome c oxidase subunit 6B1

Gene Full Name: cytochrome c oxidase subunit 6B1

Synonyms: COX VIb 1; COX6B; COX6B1; COXG

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