Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferPreservatives: 0.025% Sodium azide, 0.025% Thimerosal (merthiolate)
Constituents: 2.5% BSA, 0.45% Sodium chloride, 0.1% Dibasic monohydrogen sodium phosphate
Concentration information loading...
PurityImmunogen affinity purified
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Integration of energy
Our Abpromise guarantee covers the use of ab90668 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 1 µg/ml. Predicted molecular weight: 57 kDa.|
FunctionCytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.
PathwayEnergy metabolism; oxidative phosphorylation.
Involvement in diseaseDefects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
Defects in MT-CO1 are a cause of anemia sideroblastic acquired idiopathic (AISA) [MIM:516030]; a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria.
Defects in MT-CO1 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 MT-CO1 are associated with recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]. Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine.
Defects in MT-CO1 are a cause of deafness sensorineural mitochondrial (DFNM) [MIM:500008]. DFNM is a form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies.
Defects in MT-CO1 are a cause of colorectal cancer (CRC) [MIM:114500].
Sequence similaritiesBelongs to the heme-copper respiratory oxidase family.
Cellular localizationMitochondrion inner membrane.
- Information by UniProt
- COI antibody
- COX I antibody
- COX1 antibody
All lanes : Anti-MTCO1 antibody (ab90668) at 1 µg/ml
Lane 1 : Whole cell lysates prepared from MCF-7 cells.
Lane 2 : Whole cell lysates prepared from Hela cells.
Lane 3 : Whole cell lysates prepared from Jurkat cells.
Lane 4 : Whole cell lysates prepared from SMMC cells.
Lane 5 : Whole cell lysates prepared from Colo320 cells.
Lysates/proteins at 50 µg per lane.
All lanes : HRP-conjugated Goat anti-rabbit IgG at 1/3000 dilution
Predicted band size: 57 kDa
This product has been referenced in:
- Grover R et al. Myg1 exonuclease couples the nuclear and mitochondrial translational programs through RNA processing. Nucleic Acids Res 47:5852-5866 (2019). Read more (PubMed: 31081026) »
- Celardo I et al. dATF4 regulation of mitochondrial folate-mediated one-carbon metabolism is neuroprotective. Cell Death Differ 24:638-648 (2017). Read more (PubMed: 28211874) »