Product Details

Purity

>85% (SDS-PAGE)

Target Names

MT-CO1

Uniprot No.

P00395

Alternative Names

COI; COX I; COX1; COX1_HUMAN; COXI; Cytochrome c oxidase polypeptide I; Cytochrome c oxidase subunit 1; Cytochrome C Oxidase subunit I; Mitochondrially encoded cytochrome c oxidase I; MT CO1; MT-CO1; MTCO 1; MTCO1

Species

Homo sapiens (Human)

Protein Length

Partial

Tag Info

Tag type will be determined during the manufacturing process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.

Form

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Buffer before Lyophilization

Tris/PBS-based buffer, 6% Trehalose, pH 8.0

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet

Please contact us to get it.

Target Background

Function

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.

Gene References into Functions

TGFbeta1 reduced complex IV protein MTCO1 abundance in both myoblasts and myotubes. PMID: 29335583
We investigated A5935G, G5949A, G6081A, G6267A mutations in MT-CO1 and T9540C in MT-CO3, and alterations detected during the analysis of MT-CO gene fragments in endometrial hyperplasia. Three new alterations detected in this study (A6052G, A9545G, G9575A) were described for the first time. PMID: 28819937
The monomeric COX1 assembly intermediate accumulates demonstrating a function of COX5A in complex IV biogenesis. A potential therapeutic lead is demonstrated by showing that copper supplementation leads to partial rescue of complex IV deficiency in patient fibroblasts. PMID: 28247525
High MTCO1 expression is associated with cognitive impairment in lung cancer. PMID: 26987334
patients with primary ovarian insufficiency exhibit an increased incidence of mitochondrial cytochrome c oxidase 1 gene mutations;MT-CO1 gene mutation may be causal in the disease PMID: 26225554
studies have provided mechanistic insights into crosstalk between assembly intermediates, import processes and the synthesis of COX subunits in mitochondria, thus linking conceptually separated functions. PMID: 25663696
The m.9267G>C MT-COIII mutation was present with a nonsynonymous inherited mitochondrial homoplasmic variation MT-COI m.5913 G>A. PMID: 25701779
A novel heteroplasmic mutation was identified in MTCO1, m.7402delC, causing frameshift and a premature termination codon in a mitochondrial encephalomyopathy patient with cytochrome c oxidase deficiency. PMID: 24956508
A novel m.6307A>G mutation in the mitochondrial COXI gene in asthenozoospermic infertile men. PMID: 23712756
mitochondrial DNA mutations in COI resulting in increased reactive oxygen and reactive nitrogen generation may be involved in prostate cancer biology PMID: 23509693
Possible association of a novel missense mutation A6375G in the mitochondrial cytochrome C oxidase I gene with asthenospermia in the Tunisian population PMID: 23030649
Abeta 1-42 bound to a peptide comprising the amino-terminal region of cytochrome c oxidase subunit 1 PMID: 22927926
Both full-length and truncated COX1 proteins physically interact with AFG3L2. PMID: 22252130
the possible role of m.7445 A>G in susceptibility to aminoglycoside induced-hearing loss PMID: 21621438
Data show that homoplasmic G6709A (MT-CO1) and G14804A (MT-CYB) alterations cause amino acid changes in the highly conserved residues. PMID: 21389643
MT-CO1 has a role in moderate mental retardation and a mild exercise intolerance [case report] PMID: 18484665
Screening of a Greek deafness population for the A7445G mitochondrial DNA COI mutation. PMID: 20382059
3 single nucleotide changes in 528 bp cytochrome oxidase gene fragment from 18 myelodysplastic syndrome patients were confirmed. PMID: 18718066
C6489A missense mutation in the mitochondrial DNA (mtDNA) CO I gene encoding the cytochrome c oxidase (COX) subunit I in a 17-year-old girl with epilepsia partialis continua PMID: 12140182
study points to a role for surfeit 1(SURF1) in promoting the association of cytochrome c oxidase II with the cytochrome c oxidase I.cytochrome c oxidase subunit 4.cytochrome c oxidase subunit 5A subassembly PMID: 14607829
The expression of COX I subunits (I and III)was studied in blood platelets during aging. PMID: 14759509
High percentage of a 24.2 kb duplicated molecule was found in lymphocytes whereas the corresponding deletion dimer dominated in muscle. PMID: 15036329
A missense mutation within the mitochondrial cytochrome c oxidase I gene is associated with cancer. PMID: 16671096
Respiration dysfunction in cell with a mutation in COX I can be complementated by cell fusion with another cell carrying a mutation in cytochrome b. PMID: 16740593
Functional effects of nonsense mitochondrial DNA (mtDNA) mutations in the COXI and ND5 genes in a colorectal tumor cell line. PMID: 17341490
Cox-1 is expressed with a wide variety of levels and up-regulated significantly in endometrial cancer at the mRNA and protein levels, and may have an important role in tumor development in endometrial canceer. PMID: 18204294
the presence of one or more COI missense variants was not significantly associated with prostate cancer in African Americans PMID: 19267350
Trx2 overexpression modulates the mRNA levels of the COX1 (cytochrome oxidase subunit I) and Cytb (cytochrome b), which are known to be regulated by GR and NF-kappaB. PMID: 19570036

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Involvement in disease

Leber hereditary optic neuropathy (LHON); Mitochondrial complex IV deficiency (MT-C4D); Recurrent myoglobinuria mitochondrial (RM-MT); Deafness, sensorineural, mitochondrial (DFNM); Colorectal cancer (CRC)

Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Protein Families

Heme-copper respiratory oxidase family

Database Links

HGNC: 7419

OMIM: 114500

KEGG: hsa:4512

STRING: 9606.ENSP00000354499

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Recombinant Human Cytochrome c oxidase subunit 1 (MT-CO1), partial

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