Volume : 50 µg
Clone Number :
Aliases : COX 6B antibody; COX VIb 1 antibody; COX VIb-1 antibody; COX6B antibody; COX6B1 antibody; COXG antibody; COXVIb1 antibody; CX6B1_HUMAN antibody; Cytochrome c oxidase subunit 6B1 antibody; Cytochrome c oxidase subunit VIb antibody; Cytochrome c oxidase subunit VIb isoform 1 antibody; cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous) antibody; Cytochrome c oxidase subunit Vib polypeptide 1 antibody
Product Type : polyclonal Ab Antibody
Immunogen Species : Homo sapiens (Human)
UniProt ID : P14854
Immunogen : Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)
Raised in : Rabbit
Species Reactivity : Human
Tested Applications : ELISA
Background : 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 m µLtisystem 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.
Clonality : polyclonal Ab
Isotype : IgG
Purification Method : >95%, Protein G purified
Conj µgate : Biotin
Buffer : Preservative : 0.03% Proclin 300
Constituents : 50% Glycerol, 0.01M PBS, PH 7.4
Form : Liquid
Stroage : Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Target Names : COX6B1
Research Areas : Cancer; Tags & Cell Markers; Metabolism; Signal transduction