COX6B1 Antibody, Biotin conjugated
Produktgrößen
50 ug
£186,00
CSB-PA00395D0RB-50UG
100 ug
£334,00
CSB-PA00395D0RB-100UG
Über dieses Produkt
- SKU:
- CSB-PA00395D0RB
- Zusätzliche Namen:
- 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
- Anwendung:
- ELISA
- Buffer:
- Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
- Klonalität:
- Polyclonal
- Konjugat:
- Biotin
- Weitere Details:
- 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.
- Host:
- Rabbit
- Immunogen:
- Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)
- Isotyp:
- IgG
- Physischer Zustand:
- Liquid
- Aufreinigung:
- Protein G Purified
- Reaktivitäten:
- Human
- Versandbedingungen:
- Blue Ice
- Lagerbedingungen:
- -20[o]C/-70[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Cusabio
- Typ:
- Antibody: Polyclonal Antibody
- Datenblatt des Herstellers:COX6B1-AntibodyBiotin-conjugated-57795.html