The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 5 µg/ml.
Use a concentration of 1 µg/ml. Predicted molecular weight: 10 kDa.
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.
ICC/IF image of ab110266 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab110266, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96879, DyLight® 488 goat anti-mouse IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
Western blot - Anti-Cytochrome C Oxidase subunit VIb antibody [3F9D3D11AF6] (ab110266)
All lanes : Anti-Cytochrome C Oxidase subunit VIb antibody [3F9D3D11AF6] (ab110266) at 1 µg/ml
Lane 1 : Isolated mitochondria from Human Heart at 5 µg Lane 2 : Isolated mitochondria from Bovine Heart at 4 µg Lane 3 : Isolated mitochondria from Rat Heart at 10 µg Lane 4 : Isolated mitochondria from Mouse Heart at 10 µg
Kim SE et al. Upregulation of cytochrome c oxidase subunit 6b1 (Cox6b1) and formation of mitochondrial supercomplexes: implication of Cox6b1 in the effect of calorie restriction. Age (Dordr)37:9787 (2015).
Read more (PubMed: 25929654) »
Bourens M et al. Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase. Hum Mol GenetN/A:N/A (2014).
Read more (PubMed: 24403053) »