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Key Documents

WH0001340M1

Monoclonal Anti-COX6B1 antibody produced in mouse

Name: Monoclonal Anti-COX6B1 antibody produced in mouse
Brand: SIGMA

clone 5D3, purified immunoglobulin, buffered aqueous solution

Synonym(s):

Anti-COX6B, Anti-COXG, Anti-cytochrome c oxidase subunit Vib polypeptide 1 (ubiquitous)

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About This Item

UNSPSC Code:

12352203

NACRES:

NA.41

biological source

mouse

Quality Level

100

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

5D3, monoclonal

form

buffered aqueous solution

species reactivity

human

technique(s)

indirect ELISA: suitable
western blot: 1-5 μg/mL

isotype

IgG1κ

GenBank accession no.

NM_001863

UniProt accession no.

P14854

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... COX6B1(1340)

Immunogen

COX6B1 (NP_001854, 1 a.a. ~ 86 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGDISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

GenBank is a registered trademark of United States Department of Health and Human Services

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Storage Class Code

10 - Combustible liquids

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

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Certificates of Analysis (COA)

Lot/Batch Number

06356-5D3

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Mitochondrial complex IV deficiency, caused by mutated COX6B1, is associated with encephalomyopathy, hydrocephalus and cardiomyopathy.

Ulla Najwa Abdulhag et al.

European journal of human genetics : EJHG, 23(2), 159-164 (2014-05-02)

Isolated cytochrome c oxidase (COX) deficiency is a prevalent cause of mitochondrial disease and is mostly caused by nuclear-encoded mutations in assembly factors while rarely by mutations in structural subunits. We hereby report a case of isolated COX deficiency manifesting

Severe infantile encephalomyopathy caused by a mutation in COX6B1, a nucleus-encoded subunit of cytochrome c oxidase.

Valeria Massa et al.

American journal of human genetics, 82(6), 1281-1289 (2008-05-24)

Cytochrome c oxidase (COX) deficiency, one of the most common respiratory-chain defects in humans, has been associated with mutations in either mitochondrial DNA genes or nucleus-encoded proteins that are not part in but promote the biogenesis of COX. Mutations of

Structural organization and transcription regulation of nuclear genes encoding the mammalian cytochrome c oxidase complex.

N Lenka et al.

Progress in nucleic acid research and molecular biology, 61, 309-344 (1998-09-30)

Cytochrome c Oxidase (COX) is the terminal component of the bacterial as well as the mitochondrial respiratory chain complex that catalyzes the conversion of redox energy to ATP. In eukaryotes, the oligomeric enzyme is bound to mitochondrial innermembrane with subunits

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