COX6B1 Antibody, Biotin conjugated

Size : 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 Antibody Immunogen Species: Homo sapiens (Human) UniProt ID: P14854 Immunogen: Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA) Host: Rabbit 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 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: 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