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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
Research Area:
Cell Biology
€139 - €419
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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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