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

中文名稱：

Recombinant Human Cytochrome c oxidase subunit 1 (MT-CO1), partial
品名簡稱：

Recombinant Human MT-CO1 protein, partial
貨號：

CSB-MP015072HU
說明書：

MSDS Datasheet
規格：

￥1536
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

Greater than 90% as determined by SDS-PAGE.
生物活性：

Not Test
基因名：

MT-CO1
Uniprot No.：

P00395
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
來源：

Mammalian cell
分子量：

33.7 kDa
表達區域：

474-513aa
氨基酸序列

EAFASKRKVLMVEEPSMNLEWLYGCPPPYHTFEEPVYMKS
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標簽：

C-terminal hFc1-tagged
產品提供形式：

Liquid or 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.
緩沖液：

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
復溶：

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.
儲存條件：

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

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.
貨期：

Basically, we can dispatch the products out in 3-7 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.
Datasheet & COA：

Please contact us to get it.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. TGFbeta1 reduced complex IV protein MTCO1 abundance in both myoblasts and myotubes. PMID: 29335583
2. 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
3. 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
4. High MTCO1 expression is associated with cognitive impairment in lung cancer. PMID: 26987334
5. 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
6. 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
7. The m.9267G>C MT-COIII mutation was present with a nonsynonymous inherited mitochondrial homoplasmic variation MT-COI m.5913 G>A. PMID: 25701779
8. 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
9. A novel m.6307A>G mutation in the mitochondrial COXI gene in asthenozoospermic infertile men. PMID: 23712756
10. mitochondrial DNA mutations in COI resulting in increased reactive oxygen and reactive nitrogen generation may be involved in prostate cancer biology PMID: 23509693
11. Possible association of a novel missense mutation A6375G in the mitochondrial cytochrome C oxidase I gene with asthenospermia in the Tunisian population PMID: 23030649
12. Abeta 1-42 bound to a peptide comprising the amino-terminal region of cytochrome c oxidase subunit 1 PMID: 22927926
13. Both full-length and truncated COX1 proteins physically interact with AFG3L2. PMID: 22252130
14. the possible role of m.7445 A>G in susceptibility to aminoglycoside induced-hearing loss PMID: 21621438
15. Data show that homoplasmic G6709A (MT-CO1) and G14804A (MT-CYB) alterations cause amino acid changes in the highly conserved residues. PMID: 21389643
16. MT-CO1 has a role in moderate mental retardation and a mild exercise intolerance [case report] PMID: 18484665
17. Screening of a Greek deafness population for the A7445G mitochondrial DNA COI mutation. PMID: 20382059
18. 3 single nucleotide changes in 528 bp cytochrome oxidase gene fragment from 18 myelodysplastic syndrome patients were confirmed. PMID: 18718066
19. 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
20. 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
21. The expression of COX I subunits (I and III)was studied in blood platelets during aging. PMID: 14759509
22. High percentage of a 24.2 kb duplicated molecule was found in lymphocytes whereas the corresponding deletion dimer dominated in muscle. PMID: 15036329
23. A missense mutation within the mitochondrial cytochrome c oxidase I gene is associated with cancer. PMID: 16671096
24. 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
25. Functional effects of nonsense mitochondrial DNA (mtDNA) mutations in the COXI and ND5 genes in a colorectal tumor cell line. PMID: 17341490
26. 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
27. the presence of one or more COI missense variants was not significantly associated with prostate cancer in African Americans PMID: 19267350
28. 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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相關疾病：

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

Mitochondrion inner membrane; Multi-pass membrane protein.
蛋白家族：

Heme-copper respiratory oxidase family
數據庫鏈接：

HGNC: 7419

OMIM: 114500

KEGG: hsa:4512

STRING: 9606.ENSP00000354499