The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
備考Protein concentration is above or equal to 0.05 mg/ml.
Best use within three months from the date of receipt of this protein.
Concentration information loading...
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% 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)
機能Connects the two COX monomers into the physiological dimeric form.
関連疾患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.
配列類似性Belongs to the cytochrome c oxidase subunit 6B family.