antibody

Anti-MTCO1 antibody

MBS176987

0.1 mg

315 USD

polyclonal

rabbit

nonconjugated

western blot, immunohistochemistry, immunocytochemistry, immunohistochemistry - paraffin section

Antibody

Anti-MTCO1 antibody

[MTCO1]

[Cytochrome c oxidase subunit 1; mitochondrially encoded cytochrome c oxidase I; 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]

[Cytochrome c oxidase subunit 1; Cytochrome c oxidase subunit 1; cytochrome c oxidase subunit I; mitochondrially encoded cytochrome c oxidase I; Cytochrome c oxidase polypeptide I]

[MT-CO1]

[MT-CO1; MT-CO1; COI; MTCO1; COX1; COI; COXI; MTCO1]

COX1_HUMAN

Polyclonal

IgG

Rabbit

Human

513

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB), Immunohistochemistry (IHC) Paraffin, Immunocytochemistry (ICC)

Western Blot:. Concentration:0.1-0.5ug/ml. Tested Species: Hu. Immunohistchemistry (Paraffin-embedded Section): . Concentration: 0.5-1ug/ml. Tested Species: Hu. Antigen Retrieval: By Heat. Immunocytochemistry:. Concentration: 0.5-1ug/ml. Tested Species: Hu. Tested Species: In-house tested speceis with positive results. Predicted Species: Species predicted to be fit for the product based on sequence similarities. By Heat: Boiling the paraffin sections in 10 mM citrate buffer, pH6.0, for 20 mins is required for the staining of formalin/paraffin sections. Other applications have not been tested. Optimal dilutions should be determined by end users.

Western Blot (WB)

Immunohistochemistry (IHC)

Immunocytochemistry (ICC)

Gene Name: Mitochondrially encoded cytochrome c oxidase l. Immunogen: A synthetic peptide corresponding to a sequence at the C-terminus of human MTCO1 (501-514aa PYHTFEEPVYMKS).

Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Description: Rabbit IgG polyclonal antibody for Cytochrome c oxidase subunit 1(MT-CO1) detection. Tested with WB, IHC-P, ICC in Human. Background: Cytochrome c oxidase subunit I(CO1 or MTCO1) is 1 of 3 mitochondrial DNA(mtDNA) encoded subunits(MTCO1, MTCO2, MTCO3) of respiratory Complex IV. Complex IV is located within the mitochondrial inner membrane and is the third and final enzyme of the electron transport chain of mitochondrial oxidative phosphorylation. It is composed of 13 polypeptides. Subunits I, II, and III(MTCO1, MTCO2, MTCO3) are encoded by mtDNA while subunits IV, Va, Vb, VIa, VIb, VIc, VIIa, VIIb, VIIc, and VIII are nuclear encoded. The cytochrome c oxidase family of enzymes have 4 redox centers, 2 hemes and 2 copper centers. In mitochondrial Complex IV, the 2 hemes are a and a3 and the 2 coppers are CuA and CuB. The 2 hemes and CuB are bound to subunit I. Acin-Perez et al.(2003) identified a cell line containing single and double missense mutations in the cytochrome c oxidase(COX) subunit I gene of mouse mitochondrial DNA. And they hypothesized that deleterious mutations can arise and become predominant; cultured cells can maintain several mtDNA haplotypes at stable frequencies; the respiratory chain has little spare COX capacity; and that the size of a cavity in the vicinity of val421 in MTCO1I of animal COX may affect the function of the enzyme.

116977

P00395.1

P00395

57,041 Da

Alzheimer's Disease Pathway (83097); Alzheimer's Disease Pathway (509); Cardiac Muscle Contraction Pathway (93344); Cardiac Muscle Contraction Pathway (93992); Cytochrome C Oxidase Pathway (413434); Cytochrome C Oxidase Pathway (468345); Effects Of Nitric Oxide Pathway (920994); Electron Transport Chain Pathway (198860); Huntington's Disease Pathway (83100); Huntington's Disease Pathway (512)

COX1: Cytochrome 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. Defects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON). 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. MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis. Defects in MT-CO1 are a cause of mitochondrial complex IV deficiency (MT-C4D); 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). 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). 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). Belongs to the heme-copper respiratory oxidase family. Protein type: Membrane protein, multi-pass; Membrane protein, integral; EC 1.9.3.1; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; Mitochondrial. Chromosomal Location of Human Ortholog: -. Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Deafness, Nonsyndromic Sensorineural, Mitochondrial

0.1 mg

315 USD