Rabbit anti-Human COX6B1 Polyclonal Antibody | anti-COX6B1 antibody

COX6B1, CT (COX6B1, COX6B, Cytochrome c oxidase subunit 6B1, Cytochrome c oxidase subunit VIb isoform 1) (FITC)

Cat. Num. AAA6288134

• Gene Names: COX6B1; COXG; COX6B; COXVIb1
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: COX6B1; Polyclonal Antibody; CT (COX6B1; COX6B; Cytochrome c oxidase subunit 6B1; Cytochrome c oxidase subunit VIb isoform 1) (FITC); Cytochrome c oxidase subunit VIb isoform 1; anti-COX6B1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-COX6B1 antibody: Immunohistochemistry (IHC), FLISA, Western Blot (WB)
• Application Notes: IHC: 1:10-50; FLISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: COX6B1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 58-86 amino acids from the C-terminal region of human COX6B1.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(COX6B1 Antibody (C-term) western blot analysis in ZR-75-1,A549,NCI-H292 cell line lysates (35ug/lane).This demonstrates the COX6B1 antibody detected the COX6B1 protein (arrow).)

Immunohistochemistry (IHC)

(COX6B1 Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of COX6B1 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-COX6B1 antibody

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.

Product Categories/Family for anti-COX6B1 antibody

Antibodies; Enzymes; Cytochrome

References

Lazarou, M., et al. FEBS J. 276(22):6701-6713(2009). Massa, V., et al. Am. J. Hum. Genet. 82(6):1281-1289(2008). Sirchia, R., et al. Biol. Chem. 388(5):457-465(2007). Schmidt, T.R., et al. Gene 286(1):13-19(2002). Taanman, J.W., et al. Hum. Genet. 87(3):325-327(1991).

NCBI and Uniprot Product Information

• NCBI GI #: 4502985
• NCBI GeneID: 1340
• NCBI Accession #: NP_001854.1
• NCBI GenBank Nucleotide #: NM_001863.4
• UniProt Accession #: P14854
• NCBI Official Full Name: cytochrome c oxidase subunit 6B1
• NCBI Official Synonym Full Names: cytochrome c oxidase subunit 6B1
• NCBI Official Symbol: COX6B1
• NCBI Official Synonym Symbols: COXG; COX6B; COXVIb1
• NCBI Protein Information: cytochrome c oxidase subunit 6B1
• UniProt Protein Name: Cytochrome c oxidase subunit 6B1
• Protein Family: Cytochrome c oxidase
• UniProt Gene Name: COX6B1
• UniProt Synonym Gene Names: COX6B; COX VIb-1
• UniProt Entry Name: CX6B1_HUMAN

NCBI Description

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. [provided by RefSeq, Jan 2010]

Uniprot Description

COX6B1: Connects the two COX monomers into the physiological dimeric form. Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D); also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Belongs to the cytochrome c oxidase subunit 6B family.

Protein type: Mitochondrial; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 19q13.1

Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial intermembrane space

Molecular Function: cytochrome-c oxidase activity

Biological Process: cellular metabolic process; substantia nigra development; transmembrane transport

Disease: Mitochondrial Complex Iv Deficiency