- Catalog number
- A10285-2-Cy3
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- 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.
- Synonyms
- Pre T-cell antigen receptor alpha; pT-alpha; pTa; pT-alpha-TCR; PTCRA
- Gene Name
- pre T cell antigen receptor alpha
- Gene ID
- 1340
- UniProt
- P14854
- Host
- Rabbit
- Reactivity
- Human,Mouse,Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- 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
- Tissue Specificity
- Expressed in immature but not mature T-cells. Also found in CD34+ cells from peripheral blood, CD34+ precursors from umbilical cord blood and adult bone marrow.
- Applications
- WB,IHC,ICC,IF,Flow Cytometry
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Function
- The pre-T-cell receptor complex (composed of PTCRA, TCRB and the CD3 complex) regulates early T-cell development. .
- Components
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
- References & Citations
- 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.
- Storage Conditions
- 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.
- Calculated Molecular Weight
- 41409 MW
- Observed Molecular Weight
- 12 kDa
- Fragment
- Rabbit IgG
- Applications Notes
- Western blot, 0.25-0.5 μg/ml, Mouse, Rat<br>
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human<br>
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human<br>
Flow Cytometry (Fixed), 1-3 μg/1x10<sup>6</sup> cells, Human<br>
- Subcellular Location
- Membrane; Single-pass type I membrane protein.
- Protein Name
- Protein NDRG3