The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
COX VIb 1
Cytochrome c oxidase subunit 6B1
Cytochrome c oxidase subunit VIb
Cytochrome c oxidase subunit VIb isoform 1
Cytochrome c oxidase subunit Vib polypeptide 1
cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
Connects the two COX monomers into the physiological dimeric form.
Involvement in disease
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.
Belongs to the cytochrome c oxidase subunit 6B family.