Rabbit anti-Human MT-CO3 Polyclonal Antibody | anti-MT-CO3 antibody

MT-CO3, NT (MT-CO3, COIII, COXIII, MTCO3, Cytochrome c oxidase subunit 3, Cytochrome c oxidase polypeptide III) (FITC)

Cat. Num. AAA6322651

• Gene Names: MT-CO3; COIII; MTCO3; COX3
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: MT-CO3; Polyclonal Antibody; NT (MT-CO3; COIII; COXIII; MTCO3; Cytochrome c oxidase subunit 3; Cytochrome c oxidase polypeptide III) (FITC); Cytochrome c oxidase polypeptide III; anti-MT-CO3 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-MT-CO3 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: MT-CO3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 48-77 amino acids from the N-terminal region of human MT-CO3.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(MT-CO3 Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the MT-CO3 antibody detected the MT-CO3 protein (arrow).)

Related Product Information for anti-MT-CO3 antibody

Subunits I, II and III form the functional core of the enzyme complex.

Product Categories/Family for anti-MT-CO3 antibody

Antibodies; Enzymes; Cytochrome

References

Andrews, R.M., et al. Nat. Genet. 23 (2), 147 (1999): Anderson, S., et al. Nature 290(5806):457-465(1981) Submitted (08-JUL-2009) National Center for Biotechnology Information, NIH, Bethesda, MD 20894, USA: Kogelnik, A.M., et al. Submitted (24-AUG-2006) Mitomap.org, Center for Molecular and Mitochondrial Medicine and Genetics (MAMMAG) University of California, University of California, Irvine, Irvine, CA 92697-3940, USA: Kogelnik, A.M., et al. Submitted (18-APR-1997) Center for Molecular Medicine, Emory University School of Medicine, 1462 Clifton Road, Suite 420, Atlanta, GA 30322, USA:.

NCBI and Uniprot Product Information

• NCBI GI #: 251831113
• NCBI GeneID: 4514
• NCBI Accession #: YP_003024032.1
• NCBI GenBank Nucleotide #: NC_012920.1
• UniProt Accession #: P00414; Q14Y83; Q7GIM7
• Molecular Weight: 29,951 Da
• NCBI Official Full Name: cytochrome c oxidase subunit III
• NCBI Official Synonym Full Names: mitochondrially encoded cytochrome c oxidase III
• NCBI Official Symbol: MT-CO3
• NCBI Official Synonym Symbols: COIII; MTCO3; COX3
• NCBI Protein Information: cytochrome c oxidase III; cytochrome c oxidase subunit III
• UniProt Protein Name: Cytochrome c oxidase subunit 3
• UniProt Gene Name: MT-CO3
• UniProt Synonym Gene Names: COIII; COXIII; MTCO3
• UniProt Entry Name: COX3_HUMAN

Uniprot Description

COX3: Subunits I, II and III form the functional core of the enzyme complex. Defects in MT-CO3 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. Defects in MT-CO3 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-CO3 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. Belongs to the cytochrome c oxidase subunit 3 family.

Protein type: Mitochondrial; Membrane protein, multi-pass; Membrane protein, integral; Oxidoreductase

Chromosomal Location of Human Ortholog: -

Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Leber Optic Atrophy