Complex IV subunit 1 monoclonal antibody
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Catalog No. MS404
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$325.00 - 100 µg
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This antibody can also be purchased as part of a Sample Pack.
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UniProt Number:
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P00395
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Alternate Names:
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Cytochrome c oxidase subunit 1,
Cytochrome c oxidase polypeptide I
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Structure and Function:
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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.
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Disease Associations:
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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.
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Product Specifications
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Applications:
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Western blotting, Immunocytochemistry, Immunohistochemistry, In-Cell ELISA
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Species Reactivity:
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human, bovine, mouse, rat, zebrafish, C. elegans, oyster
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Host Species:
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mouse
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Isotype:
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IgG2a, κ
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Clone ID:
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1D6E1A8
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Concentration:
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1.0 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Suggested Working Concentration:
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1 µg/mL for Western blotting
5 µg/mL for Immunocytochemistry
8 µg/mL for In-Cell ELISA (0.8 µg/well)
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Storage Conditions:
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Store at 4°C. Do not freeze.
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Country of Origin:
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USA
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WB Images
(click to enlarge)
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.
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ICC Images
(click to enlarge)
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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IHC Images
(click to enlarge)
Figure 3. Skeletal muscle immunohistochemistry using MS404- fixed frozen tissue sections from a patient with a single large deletion of the mtDNA show a mosaic of complex IV positive and complex IV negative fibers. Image kindly provided by Dr. J. Murphy and D. Turnbull, Mitochondrial Research Group, Newcastle University.
(click to enlarge)
Figure 4. Colon Immunohistochemistry - 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. 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)
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Published Studies Using This Product:
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Misiak et al., 2010. Brain region-specific vulnerability of astrocytes in response to 3-nitropropionic acid is mediated by cytochrome c oxidase isoform expression.
Brys et al., 2010. Disruption of insulin signalling preserves bioenergetic competence of mitochondria in ageing Caenorhabditis elegans.
Kotarsky et al., 2010. Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation.
Safdar et al., 2010. Aberrant mitochondrial homeostasis in the skeletal muscle of sedentary older adults.
Crawford et al., 2010. Naturally occurring R225W mutation of the gene encoding AMP-activated protein kinase (AMPK)gamma(3) results in increased oxidative capacity and glucose uptake in human primary myotubes.
Patel et al., 2010. Caloric restriction shortens lifespan through an increase in lipid peroxidation, inflammation and apoptosis in the G93A mouse, an animal model of ALS.
Nadanaciva et al., 2010. High-Content Screening for Compounds That Affect mtDNA-Encoded Protein Levels in Eukaryotic Cells.
Fogal et al., 2010. Mitochondrial p32 Protein is a Critical Regulator of Tumor Metabolism via Maintenance of Oxidative Phosphorylation.
Perkins et al., 2010. The micro-architecture of mitochondria at active zones: electron tomography reveals novel anchoring scaffolds and cristae structured for high-rate metabolism.
Khalimonchuk et al., 2009. Formation of the Redox Cofactor Centers during Cox1 Maturation in Yeast Cytochrome Oxidase.
López-Royuela et al., 2009. Bim is the key mediator of glucocorticoid-induced apoptosis and of its potentiation by rapamycin in human myeloma cells.
Beck Jřrgensen et al., 2009. Reduced AMP-activated protein kinase activity in mouse skeletal muscle does not exacerbate the development of insulin resistance with obesity.
Perez-Martinez et al., 2009. Dual functions of Mss51 couple synthesis of Cox1 to assembly of cytochrome c oxidase in Saccharomyces cerevisiae mitochondria.
Gómez et al., 2009. Supercomplexes of the mitochondrial electron transport chain decline in the aging rat heart.
Rackham et al., 2009. Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells.
Lin et al., 2009. Mechanisms of resveratrol-induced platelet apoptosis.
Oswald et al., 2009. Knockdown of human COX17 affects assembly and supramolecular organization of cytochrome c oxidase.
Lastra et al., 2009. Direct renin inhibition improves systemic insulin resistance and skeletal muscle glucose transport in a transgenic rodent model of tissue renin overexpression.
Hanein et al., 2009. TMEM126A, encoding a mitochondrial protein, is mutated in autosomal-recessive nonsyndromic optic atrophy.
Nadanaciva et al., 2009. Lateral-flow immunoassay for detecting drug-induced inhibition of mitochondrial DNA replication and mtDNA-encoded protein synthesis.
Tang et al., 2009. hNOA1 interacts with complex I and DAP3 and regulates mitochondrial respiration and apoptosis.
Vempati et al., 2009. Lack of cytochrome c in mouse fibroblasts disrupts assembly/stability of respiratory complexes I and IV.
Alves et al., 2009. Acetyl-L-carnitine provides effective in vivo neuroprotection over 3,4-methylenedioximethamphetamine-induced mitochondrial neurotoxicity in the adolescent rat brain.
Song et al., 2009. Analysis of protein expression and compartmentalization in retinal neurons using serial tangential sectioning of the retina.
Suarez et al., 2008. Alterations in mitochondrial function and cytosolic calcium induced by hyperglycemia are restored by mitochondrial transcription factor A in cardiomyocytes.
Habibi et al., 2008. Renin inhibition attenuates insulin resistance, oxidative stress, and pancreatic remodeling in the transgenic Ren2 rat.
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.
Natoli et al., 2008. Expression and role of the early-response gene Oxr1 in the hyperoxia-challenged mouse retina.
Jones et al., 2008. Caffeine stimulates cytochrome oxidase expression and activity in the striatum in a sexually dimorphic manner.
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-1alpha integrates insulin signaling, mitochondrial regulation, and bioenergetic function in skeletal muscle.
Putignani et al., 2008. Alteration of expression levels of the oxidative phosphorylation system (OXPHOS) in breast cancer cell mitochondria.
Pierrel et al., 2008. Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of Cox1.
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.
Yu et al., 2008. Differential loss of cytochrome-c oxidase subunits in ischemia-reperfusion injury: exacerbation of COI subunit loss by PKC-epsilon inhibition.
Son et al., 2008. Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein.
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.
Devi et al., 2008. Mitochondrial import and accumulation of alpha-synuclein impair complex I in human dopaminergic neuronal cultures and Parkinson disease brain.
Zielonka et al., 2008. Cytochrome c-mediated oxidation of hydroethidine and mito-hydroethidine in mitochondria: identification of homo- and heterodimers.
Desquiret et al., 2008. Mitochondrial effects of dexamethasone imply both membrane and cytosolic-initiated pathways in HepG2 cells.
Baden et al., 2007. Early developmental pathology due to cytochrome c oxidase deficiency is revealed by a new zebrafish model.
Jha et al., 2007. Crucial role of apoptosis in the resolution of experimental autoimmune anterior uveitis.
Hawkins et al., 2007. G protein-coupled receptor Ca2+-linked mitochondrial reactive oxygen species are essential for endothelial/leukocyte adherence.
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.
Rea et al., 2007. Relationship between mitochondrial electron transport chain dysfunction, development, and life extension in Caenorhabditis elegans.
Pravenec et al., 2007. Direct linkage of mitochondrial genome variation to risk factors for type 2 diabetes in conplastic strains.
Song et al., 2007. Compartment-specific phosphorylation of phosducin in rods underlies adaptation to various levels of illumination.
Li et al., 2007. An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondria.
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.
Ventura et al., 2007. Caenorhabditis elegans mitochondrial mutants as an investigative tool to study human neurodegenerative diseases associated with mitochondrial dysfunction.
Scheper et al., 2007. Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation.
Jung et al., 2007. Ethanol withdrawal posttranslationally decreases the activity of cytochrome c oxidase in an estrogen reversible manner.
Dell'agnello et al., 2007. Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice.
Boesze-Battaglia et al., 2007. The tetraspanin protein peripherin-2 forms a complex with melanoregulin, a putative membrane fusion regulator.
Callahan et al., 2007. Diaphragm and cardiac mitochondrial creatine kinases are impaired in sepsis.
Li et al., 2006. Cytochrome c oxidase subunit IV is essential for assembly and respiratory function of the enzyme complex.
Wang et al., 2006. Human mitochondrial DNA nucleoids are linked to protein folding machinery and metabolic enzymes at the mitochondrial inner membrane.
Sundaram et al., 2006. ATP-binding cassette transporters are required for efficient RNA interference in Caenorhabditis elegans.
Pye et al., 2006. Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.
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.
Cisar et al., 2004. Differential expression of mitochondrial electron transport chain proteins in cardiac tissues of broilers from pulmonary hypertension syndrome-resistant and -susceptible lines.
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