Product nameAnti-MTCO1 antibody [EPR19642] - BSA and Azide free
See all MTCO1 primary antibodies
DescriptionRabbit monoclonal [EPR19642] to MTCO1 - BSA and Azide free
Tested applicationsSuitable for: Flow Cyt, WB, IHC-P, ICC/IFmore details
Species reactivityReacts with: Human
Synthetic peptide within Human MTCO1 aa 450 to the C-terminus. The exact sequence is proprietary.
Database link: P00395
Ab251408 is the carrier-free version of ab203917. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
ab251408 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Concentration information loading...
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Integration of energy
Our Abpromise guarantee covers the use of ab251408 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Flow Cyt||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration. Detects a band of approximately 37 kDa (predicted molecular weight: 57 kDa).|
|IHC-P||Use at an assay dependent concentration. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.|
|ICC/IF||Use at an assay dependent concentration.|
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
ab251408 has not yet been referenced specifically in any publications.