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Figure 1. Isolated mitochondria from human heart (lane 1), bovine heart (lane 2), rat heart (lane 3), and mouse heart (lane 4) detected with (MS404) anti-subunit 1 antibody. Extra bands in the mouse sample (lane 4) are due to the reaction of the IgG-specific goat anti-mouse secondary antibody with residual mouse blood in the heart tissue, as it is very difficult to entirely remove the blood from these small organs.

Figure 2. Image of the CcoI antibody (green) used to stain MELAS (myoclonus, epilepsy, lactic acidosis and stroke like episodes) where a mutation in a mitochondrially-encoded tRNA gene alters the biogenesis of Cco. A noted difference in staining can be seen between cells which have the CcoI subunit and those which either have little to none of the subunit present. The antibody was used at 7.5 ug/mL of anti-Cco subunit 1 mAb (MS404) in 10% goat serum, overnight at 4°C, when testing fibroblasts or other adherent cells.

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Figure 3. Skeletal Muscle Immunohistochemistry – Tissue sections from a patient with a single large deletion of the mtDNA. Kindly provided by Dr. J. Murphy and D. Turnbull, Mitochondrial Research Group, Newcastle University.

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Figure 4. Colon Immunohistochemistry – Tissue sections from a normal ageing patient. Kindly provided by Dr. L. Greaves and D. Turnbull, Mitochondrial Research Group, Newcastle University. For more details, see Taylor et al., J. Clin. Invest. 112:1351-1360 (2003)

Complex IV subunit 1 monoclonal antibody

Catalog No. MS404

$295.00 - 100 µg

UniProt Number: P00395
Alternate Names: Cytochrome c oxidase subunit 1, Cytochrome c oxidase polypeptide I
Structure and Function: Cytochrome c oxidase is the fourth complex in the respiratory chain and is responsible for catalyzing the conversion of O2 to H2O. Subunit 1 of the cytochrome c oxidase complex combines with two other subunits (2 and 3) to form a core protein structure that performs many of the functions of the enzyme. The heme A group of this subunit works with the copper A group of subunit 2 to transfer electrons from cytochrome c to the metallic center in complex IV where heme A3 and copper B reside.
Disease Associations: Defects in subunit 1 result in a number of pathologies including Leber hereditary optic neuropathy (LHON), acquired idiopathic sidereoblastic anemia (AISA), cytochrome c oxidase deficiency (COX deficiency), and recurrent myogloninuria. LHON is a maternally inherited pathology that affects mainly young men, causing sudden blindness in both eyes as a result of retinal degeneration. Other symptoms of this disorder include problems with cardiac conduction and neurological deficits. AISA causes a build up of iron stores in mitochondria, due to the inability to properly form heme. COX deficiency results in a wide range of symptoms from local myopathy to multiple system pathologies that begin between infancy and adulthood. Myoglobinuria is a condition that results in myoglobin levels in the urine.

Product Specifications
Applications: Western blotting, Immunocytochemistry, and Immunohistochemistry
Species Reactivity: human, bovine, mouse, rat, zebrafish, C. elegans, and oyster
Host Species: mouse
Isotype: IgG2a, k
Clone ID: 1D6E1A8
Immunogen: Human Complex IV subunit I, from SDS-PAGE
Concentration: 1.0 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
Suggested Working Concentration: 1 µg/mL for Western blotting, 5 µg/mL for Immunocytochemistry
Storage Conditions: 4°C
Country of Origin: USA
Citations: Desquiret et al., 2008. Mitochondrial effects of dexamethasone imply both membrane and cytosolic-initiated pathways in HepG2 cells.

Greiss et al., 2008. C. elegans SIR-2.1 translocation is linked to a proapoptotic pathway parallel to cep-1/p53 during DNA damage-induced apoptosis.

Rorbach et al., 2008. The human mitochondrial ribosome recycling factor is essential for cell viability.

Mabalirajan et al., 2008. Mitochondrial Structural Changes and Dysfunction Are Associated with Experimental Allergic Asthma.

Lynn et al., 2008. SIRT2 is a negative regulator of anoxia–reoxygenation tolerance via regulation of 14-3-3 zeta and BAD in H9c2 cells.

Pagel-Langenickel et al., 2008. PGC-1 {alpha} Integrates Insulin Signaling, Mitochondrial Regulation, and Bioenergetic Function in Skeletal Muscle.

Genova et al., 2008. Is supercomplex organization of the respiratory chain required for optimal electron transfer activity?.

Johnson et al., 2008. Cardiac Cytochrome c Oxidase Activity and Contents of Subunits 1 and 4 Are Altered in Offspring by Low Prenatal Copper Intake by Rat Dams.

Whaley-Connell et al., 2008. Effect of renin inhibition and AT1R blockade on myocardial remodeling in the transgenic Ren2 rat.

Pierrel et al., 2008. Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis facilitating the maturation of Cox1.

Son et al., 2008. Isolated Cytochrome c Oxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein.

Yu et al., 2008. Differential loss of cytochrome-c oxidase subunits in ischemia-reperfusion injury: exacerbation of COI subunit loss by PKC-e inhibition.

Zielonka et al., 2008. Cytochrome c-mediated oxidation of hydroethidine and mito-hydroethidine in mitochondria: Identification of homo-and heterodimers.

Devi et al., 2008. Mitochondrial Import and Accumulation of a-Synuclein Impairs complex I in Human Dopaminergic Neuronal Cultures and Parkinson’s disease Brain.

Bastin et al., 2008. Activation of peroxisome proliferator activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components.

Baden et al., 2007. Early Developmental Pathology Due to Cytochrome c Oxidase Deficiency Is Revealed by a New Zebrafish Model.

Rea et al., 2007. Relationship Between Mitochondrial Electron Transport Chain Dysfunction, Development, and Life Extension in Caenorhabditis elegans.

Margineantu et al., 2007. Hsp90 Inhibition Decreases Mitochondrial Protein Turnover.

Pierrel et al., 2007. Coa1 links the Mss51 post-translational function to Cox1 cofactor insertion in cytochrome c oxidase assembly.

Song et al., 2007. Compartment-specific Phosphorylation of Phosducin in Rods Underlies Adaptation to Various Levels of Illumination.

Pravenec et al., 2007. Direct linkage of mitochondrial genome variation to risk factors for type 2 diabetes in conplastic strains.


Li et al., 2007. An Assembled Complex IV Maintains the Stability and Activity of Complex I in Mammalian Mitochondria.

Ventura et al., 2007. Caenorhabditis elegans mitochondrial mutants as an investigative tool to study human neurodegenerative diseases associated with mitochondrial dysfunction.

Park et al., 2007. Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation.

Jung et al., 2007. Ethanol withdrawal posttranslationally decreases the activity of cytochrome c oxidase in an estrogen reversible manner.

Scheper et al., 2007. Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation.

Dell’Agnello et al., 2007. Increased longevity and refractoriness to Ca2+-dependent neurodegeneration in Surf1 knockout mice.

Jha et al., 2007. Crucial Role of Apoptosis in the Resolution of Experimental Autoimmune Anterior Uveitis.

Boesze-Battaglia et al., 2007. The Tetraspanin Protein Peripherin-2 Forms a Complex with Melanoregulin, a Putative Membrane Fusion Regulator.

Li et al., 2006. Cytochrome c oxidase subunit IV is essential for assembly and respiratory function of the enzyme complex.

Pye et al., 2006. Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.

Sundaram et al., 2006. ATP-binding Cassette Transporters Are Required for Efficient RNA Interference in Caenorhabditis elegans.

Wang et al., 2006. Human Mitochondrial DNA Nucleoids Are Linked to Protein Folding Machinery and Metabolic Enzymes at the Mitochondrial Inner Membrane.

Grad et al., 2006. Riboflavin enhances the assembly of mitochondrial cytochrome c oxidase in C. elegans NADH-ubiquinone oxidoreductase mutants.

Ogilvie et al., 2005. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy.

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