Anti-MTCO1 [1D6E1A8] antibody (ab14705)
- Product nameAnti-MTCO1 [1D6E1A8] antibodySee all MTCO1 primary antibodies ...
- DescriptionMouse monoclonal [1D6E1A8] to MTCO1
- SpecificityIn mouse liver lysate a specific band below 37 kDa was detected.
- Tested applicationsICC/IF, IHC-P, WB, ICC, Flow Cyt, IHC-Fr more details
- Species reactivityReacts with: Mouse, Rat, Cow, Human, Caenorhabditis elegans, Zebrafish, Rhesus monkey
Purified mitochondrial Complex IV subunit I (Human).
- Positive control
- Human heart mitochondria. This antibody gave a positive result in IHC in the following FFPE tissue: Human normal colon. In Flow Cytometry, this antibody gave a positive signal in methanol fixed/Tween permeabilised HEK293 cells.
- General notes
Samples that are to be used in Western blotting with this antibody should NOT be boiled before loading onto the gel. Boiling of the sample will cause a loss of signal in Western blotting (see WB image on ab110413).
- Storage instructionsStore at +4°C.
- Storage bufferPreservative: 0.02% Sodium azide
Constituents: HEPES, Sodium chloride
- Concentration information loading...
- PurityAmmonium Sulphate Precipitation
- Purification notesNear homogeneity as judged by SDS-PAGE. The antibody was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
- Clonality Monoclonal
- Clone number1D6E1A8
- Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Integration of energy
Our Abpromise guarantee covers the use of ab14705 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||ICC/IF: Use at an assay dependent dilution.|
|IHC-P||IHC-P: Use a concentration of 5 µg/ml.|
|WB||WB: Use a concentration of 0.5 µg/ml. Detects a band of approximately 40 kDa (predicted molecular weight: 57 kDa).|
|ICC||ICC: Use a concentration of 5 µg/ml.|
|Flow Cyt||Flow Cyt: Use 1µg for 106 cells.|
|IHC-Fr||IHC-Fr: Use at an assay dependent dilution.|
- 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.
- Entrez Gene: 2565700 Caenorhabditis elegans
- Entrez Gene: 281919 Cow
- Entrez Gene: 4512 Human
- Entrez Gene: 17708 Mouse
- Entrez Gene: 26195 Rat
- Entrez Gene: 140539 Zebrafish
- Omim: 516030 Human
- SwissProt: P00396 Cow
- COI antibody
- COX I antibody
- COX1_HUMAN antibody
- COXI antibody
- Cytochrome c oxidase polypeptide I antibody
- Cytochrome c oxidase subunit 1 antibody
- Cytochrome C Oxidase subunit I antibody
- Mitochondrially encoded cytochrome c oxidase I antibody
- MT CO1 antibody
- MT-CO1 antibody
- MTCO 1 antibody
- MTCO1 antibody
Anti-MTCO1 [1D6E1A8] antibody images
ab14705 staining MTCO1 - Mitochondrial Marker in pig retinal pigment epithelial cells by ICC/IF (Immunocytochemistry/immunofluorescence). Cells were fixed with paraformaldehyde, permeabilized with 0.5% TX-100 and blocked with 5% serum for 20 minutes at 25°C. Samples were incubated with primary antibody (1/2000 in 1% goat serum, 0.1% TX100; PBS) for 16 hours at 4°C. An Alexa Fluor®546-conjugated Goat polyclonal to mouse IgG, dilution 1/500, was used as secondary antibody.
ab14705 staining MTCO1 - Mitochondrial Marker in rat cerebellum primary cells by ICC/IF (Immunocytochemistry/immunofluorescence). Cells were fixed with paraformaldehyde, permeabilized with 0.5% TX-100 and blocked with 5% serum for 20 minutes at 25°C. Samples were incubated with primary antibody (1/2000 in 1% goat serum, 0.1% TX100; PBS) for 16 hours at 4°C. An Alexa Fluor®546-conjugated Goat polyclonal to mouse IgG, dilution 1/5000, was used as secondary antibody.
Overlay histogram showing HEK293 cells stained with ab14705 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab14705, 1µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 1µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed.
All lanes : Anti-MTCO1 [1D6E1A8] antibody (ab14705)
Lane 1 : Isolated mitochondria from Human heart at 5 µg
Lane 2 : Isolated mitochondria from Bovine heart at 1 µg
Lane 3 : Isolated mitochondria from Rat heart at 10 µg
Lane 4 : Isolated mitochondria from Mouse heart at 5 µg
Goat Anti-Mouse IgG
Predicted band size : 57 kDa
Observed band size : 40 kDa (why is the actual band size different from the predicted?)
Additional bands at : 70 kDa. We are unsure as to the identity of these extra bands.
ab14705 staining MTCO1 in skeletal muscle tissue by Immunohistochemistry (Frozen sections). Tissue sections were from from a patient with a single large deletion of the mtDNA and show a mosaic of complex IV positive and complex IV negative fibers.
ab14705 staining MTCO1 in Human colon tissue by Immunohistochemistry (Frozen sections). Tissue sections from a normal ageing patient show complex IV negative crypts due to clonal expansion of colonic stem cells bearing mutations in the mtDNA-encoded gene for complex IV.
IHC image of MTCO1 staining in human normal colon formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab14705, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
ab14705 staining rat pancreas sections by IHC-P. The tissue was fixed with formaldehyde and a heat mediated antigen retrival step was performed with citric acid pH 6. Blocking of the sample was done with 1% BSA for 10 minutes at 21°C, followed by staining with ab14705 at 1/1000 in TBS/BSA/azide for 2h at 21°C. A biotinylated goat anti-mouse polyclonal antibody at 1/200 was used as the secondary antibody. Positivity in exocrine glands appears to be intense at the cytplasm of adjacent cells. The cells of the Islet of Langerhan to the right have a diffuse, punctate positivity.
References for Anti-MTCO1 [1D6E1A8] antibody (ab14705)
This product has been referenced in:
- Kumarasamy S et al. Construction of two novel reciprocal conplastic rat strains and characterization of cardiac mitochondria. Am J Physiol Heart Circ Physiol 304:H22-32 (2013). WB ; Rat . Read more (PubMed: 23125210) »
- van Bon BW et al. CEP89 is required for mitochondrial metabolism and neuronal function in man and fly. Hum Mol Genet 22:3138-51 (2013). Read more (PubMed: 23575228) »