COX6B1 Antibody - Cat. #: CSB-PA00395A0Rb

SKU: CSB-PA00395A0Rb

Size :50ug

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 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, IF; Recommended dilution: IF:1:50-1:200

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 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.

Clonality:Polyclonal

Isotype:IgG

Purification Method:>95%, Protein G purified

Conjugate:Non-conjugated

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