Recombinant Human Cytochrome c oxidase subunit 3 (MT-CO3)

Code CSB-CF015074HU
MSDS
Size Pls inquire
Source in vitro E.coli expression system
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Product Details

Target Names
MT-CO3
Uniprot No.
Alternative Names
MT-CO3; COIII; COXIII; MTCO3; Cytochrome c oxidase subunit 3; Cytochrome c oxidase polypeptide III
Species
Homo sapiens (Human)
Expression Region
1-261
Target Protein Sequence
MTHQSHAYHMVKPSPWPLTGALSALLMTSGLAMWFHFHSMTLLMLGLLTNTLTMYQWWRD VTRESTYQGHHTPPVQKGLRYGMILFITSEVFFFAGFFWAFYHSSLAPTPQLGGHWPPTG ITPLNPLEVPLLNTSVLLASGVSITWAHHSLMENNRNQMIQALLITILLGLYFTLLQASE YFESPFTISDGIYGSTFFVATGFHGLHVIIGSTFLTICFIRQLMFHFTSKHHFGFEAAAW YWHFVDVVWLFLYVSIYWWGS
Protein Length
full length protein
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
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.

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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
  1. There was a significant association of cytochrome c oxidase III 15bp deletion with human male infertility. PMID: 26712170
  2. this is the first study showing to demonstrate that aa72-117 in HBx is the key region for binding with COXIII. PMID: 25483779
  3. A novel COIII gene m.9588G>A mutation was found in the mtDNA sperm's in all asthenozoospermic patients and was absent in the normozoospermic and in fertile men. PMID: 24550096
  4. novel heteroplasmic mutation m.9276G>C in the mitochondrial COIII gene, detected in mtDNA extracted from leukocytes of a mother and her two daughters indicating that this mutation is maternally transmitted PMID: 25701779
  5. Lack of Cox3 limits the biosynthesis of COX but does not alter the structure of the enzyme. PMID: 25588698
  6. A novel heteroplasmic missense mitochondrial mutation (m.9387 G>A) in COXIII gene found in 3 asthenospermic patients from Tunisia. PMID: 23645088
  7. A novel COX III subunit single base pair deletion is associated with a high number (45%) of COX-negative muscle fibers in a patient with severe rhabdomyolysis but no family history of neuromuscular disorder. PMID: 21163656
  8. a novel heteroplasmic missense mitochondrial mutation at nucleotide 9478 in COX3 gene in patients with Leigh syndrome PMID: 20525945
  9. The total mtDNA major arc deletion level was greater than the common deletion level in COX 3-deficient spiral ganglion cells. PMID: 20085441
  10. Data show that both normal and mutant alpha-synuclein specifically interact with the mitochondrial complex IV enzyme, cytochrome C oxidase (COX). PMID: 12059041
  11. Mutations in mtDNA-encoded cytochrome c oxidase subunit III genes causing isolated myopathy or severe encephalomyopathy. PMID: 16288875
  12. a MELAS syndrome Korean family harbor homoplasmic missense mutation in mitochondrial genom, which confers pathogenicity (Phe251Leu) PMID: 18587274

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Involvement in disease
Leber hereditary optic neuropathy (LHON); Mitochondrial complex IV deficiency (MT-C4D); Recurrent myoglobinuria mitochondrial (RM-MT)
Subcellular Location
Mitochondrion inner membrane; Multi-pass membrane protein.
Protein Families
Cytochrome c oxidase subunit 3 family
Database Links

HGNC: 7422

OMIM: 220110

KEGG: hsa:4514

STRING: 9606.ENSP00000354982

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