MT-CO1 sirna

MT-CO1 siRNA (Human)

Cat. Num. AAA8239359

• Gene Names: MT-CO1; COI; MTCO1; COX1
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: MT-CO1; MT-CO1 siRNA (Human); COI; COXI; MTCO1; Cytochrome c oxidase subunit 1; Cytochrome c oxidase polypeptide I; MT-CO1 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: MT-CO1 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 513

• Applicable Applications for MT-CO1 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommend transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human MT-CO1 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Ships on blue ice. Store at -20°C for one year.

Related Product Information for MT-CO1 sirna

siRNA to inhibit MT-CO1 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 251831109
• NCBI GeneID: 4512
• NCBI Accession #: YP_003024028.1
• NCBI GenBank Nucleotide #: NC_012920.1
• UniProt Accession #: P00395; Q34770
• Molecular Weight: 57,041 Da
• NCBI Official Full Name: cytochrome c oxidase subunit I (mitochondrion)
• NCBI Official Synonym Full Names: mitochondrially encoded cytochrome c oxidase I
• NCBI Official Symbol: MT-CO1
• NCBI Official Synonym Symbols: COI; MTCO1; COX1
• NCBI Protein Information: cytochrome c oxidase subunit I
• UniProt Protein Name: Cytochrome c oxidase subunit 1
• UniProt Gene Name: MT-CO1
• UniProt Synonym Gene Names: COI; COXI; MTCO1
• UniProt Entry Name: COX1_HUMAN

Uniprot Description

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: Oxidoreductase; Membrane protein, integral; Mitochondrial; EC 1.9.3.1; Energy Metabolism - oxidative phosphorylation; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: -

Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Deafness, Nonsyndromic Sensorineural, Mitochondrial