COX6B1 Antibody | CSB-PA00395A0Rb

COX6B1 Antibody | CSB-PA00395A0Rb | Cusabio

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Product Type: Polyclonal Antibody

Target Names: COX6B1

Aliases: Cytochrome c oxidase subunit 6B1 (Cytochrome c oxidase subunit VIb isoform 1) (COX VIb-1), COX6B1, COX6B

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.

Isotype: IgG

Conjugate: Non-conjugated

Clonality: Polyclonal

Uniport ID: P14854

Host Species: Rabbit

Species Reactivity: Human

Immunogen: Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)

Immunogen Species: Human

Applications: ELISA, IF

Tested Applications: ELISA, IF; Recommended dilution: IF:1:50-1:200

Purification Method: >95%, Protein G purified

Dilution Ratio1: ELISA:1:2000-1:10000

Dilution Ratio2: IF:1:50-1:200

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

Form: Liquid

Storage: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Initial Research Areas: Tags & Cell Markers

Research Areas: Cancer;Tags & Cell Markers;Metabolism;Signal transduction