Product nameAnti-MTCO1 antibody [11D8B7]
See all MTCO1 primary antibodies
DescriptionMouse monoclonal [11D8B7] to MTCO1
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Saccharomyces cerevisiae
Does not react with: Mouse, Rat, Human
Full length protein. This information is considered to be commercially sensitive.
- Mitochondria from yeast membrane extract
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesNear homogeneity as judged by SDS-PAGE. ab110270 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation
Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Integration of energy
Our Abpromise guarantee covers the use of ab110270 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 3 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
- SwissProt: P00401 Saccharomyces cerevisiae
- COI antibody
- COX I antibody
- COX1 antibody
This product has been referenced in:
- Taylor NG et al. The Assembly Factor Pet117 Couples Heme a Synthase Activity to Cytochrome Oxidase Assembly. J Biol Chem 292:1815-1825 (2017). Read more (PubMed: 27998984) »
- Swenson S et al. Analysis of Oligomerization Properties of Heme a Synthase Provides Insights into Its Function in Eukaryotes. J Biol Chem 291:10411-25 (2016). Read more (PubMed: 26940873) »