Anti-COX6B1 Antibody (CAB2228)
- SKU:
- CAB2228
- Product type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- COX6B1
- COX6B
- COXG
- COXVIb1
- cytochrome c oxidase subunit 6B1
Frequently bought together:
Description
| Product Name: | COX6B1 Rabbit mAb |
| Product Code: | CAB2228 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | COX6B1, COX6B, COXG, COXVIb1, cytochrome c oxidase subunit 6B1 |
| Applications: | WB, IHC |
| Reactivity: | Human |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human COX6B1. |
| Applications: | WB, IHC |
| Recommended Dilutions: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human |
| Positive Samples: | 293T, SH-SY5Y |
| Immunogen: | A synthesized peptide derived from human COX6B1. |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 1340 |
| Uniprot: | P14854 |
| Cellular Location: | Mitochondrion intermembrane space |
| Calculated MW: | 10kDa |
| Observed MW: | 10KDa |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | 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 |
| NCBI Summary: | 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 Code: | P14854 |
| NCBI GenInfo Identifier: | 117115 |
| NCBI Gene ID: | 1340 |
| NCBI Accession: | P14854.2 |
| UniProt Related Accession: | P14854 |
| Molecular Weight: | 10kDa |
| NCBI Full Name: | Cytochrome c oxidase subunit 6B1 |
| NCBI 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 |
| UniProt Synonym Protein Names: | Cytochrome c oxidase subunit VIb isoform 1; COX VIb-1 |
| Protein Family: | Cytochrome c oxidase |
| UniProt Gene Name: | COX6B1 |
| UniProt Entry Name: | CX6B1_HUMAN |
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