COX6B1 Antibody, HRP conjugated

Description

Antibody Type

Polyclonal Antibody

Species

Human

Uniprot ID

P14854

Immunogen

Recombinant human Cytochrome c oxidase subunit 6B1 protein (1-86AA)

Raised In

Rabbit

Species Reactivity

Human

Tested Applications

ELISA;Not yet tested in other applications.

Background / Function

Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; 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.

Isotype

IgG

Conjugate

HRP

Storage Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Form

Liquid

Storage

Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.

Purity

Caprylic Acid Ammonium Sulfate Precipitation purified

Modification

Cytochrome c oxidase subunit 6B1 protein

Research

Metabolism

Literature

[1] “Nucleotide sequence of cDNA encoding subunit VIb of human cytochrome c oxidase.”Taanman J.-W., Schrage C., Ponne N.J., Bolhuis P.A., de Vries H., Agsteribbe E.Nucleic Acids Res. 17:1766-1766(1989) [2] “Isolation of cDNAs encoding subunit VIb of cy

Additional information

Size	50μg, 100μg