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. Detects a band of approximately 49 kDa (predicted molecular weight: 49 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Converts protoheme IX and farnesyl diphosphate to heme O.
Involvement in disease
Defects in COX10 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. Defects in COX10 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
ICC/IF image of ab84053 stained HepG2 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 ab84053 at 5µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit (ab96899) 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 - COX10 antibody (ab84053)
Anti-COX10 antibody (ab84053) at 1 µg/ml (in 5% skim milk / PBS buffer) + Human fetal lung lysate at 10 µg
Secondary HRP conjugated anti-Rabbit IgG at 1/50000 dilution
Predicted band size: 49 kDa Observed band size: 49 kDa
Western blot - Anti-COX10 antibody (ab84053)
All lanes : Anti-COX10 antibody (ab84053) at 1/1000 dilution
Lane 1 : Isolated mitochondria from wild type mice at 15 µg Lane 2 : Isolated mitochondria from wild type mice at 30 µg