Recombinant Human Protein SCO1 homolog, mitochondrial (SCO1), partial

Code CSB-YP020852HU
MSDS
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Source Yeast
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Code CSB-EP020852HU
MSDS
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Source E.coli
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Code CSB-EP020852HU-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP020852HU
MSDS
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Source Baculovirus
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Code CSB-MP020852HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
SCO1
Uniprot No.
Alternative Names
Cytochrome oxidase deficient homolog; Cytochrome oxidase deficient homolog 1; Protein SCO1 homolog mitochondrial; Protein SCO1 homolog, mitochondrial; SCO (cytochrome oxidase deficient yeast) homolog 1; SCO cytochrome oxidase deficient homolog 1 (yeast); SCO cytochrome oxidase deficient homolog 1; sco1; SCO1_HUMAN; SCOD1
Species
Homo sapiens (Human)
Expression Region
132-301aa
Target Protein Sequence
GKPLLGGPFSLTTHTGERKTDKDYLGQWLLIYFGFTHCPDVCPEELEKMIQVVDEIDSITTLPDLTPLFISIDPERDTKEAIANYVKEFSPKLVGLTGTREEVDQVARAYRVYYSPGPKDEDEDYIVDHTIIMYLIGPDGEFLDYFGQNKRKGEIAASIATHMRPYRKKS
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.
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
Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2. Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1.
Gene References into Functions
  1. Results find that COA6 associates with COX2 and is crucial for its maturation and complex IV biogenesis. Also, COA6 interacts with the copper chaperone SCO1 which indicates that COA6 is intrinsically involved in the copper delivery process for COX2. PMID: 26160915
  2. Sco1 is a metallochaperone that selectively transfers Cu(I) ions based on loop recognition, whereas Sco2 is a copper-dependent thiol reductase of the cysteine ligands in the oxidase. PMID: 26351686
  3. COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase. PMID: 24403053
  4. COX19 is necessary for the transduction of a SCO1-dependent mitochondrial redox signal that regulates ATP7A-mediated cellular copper efflux. PMID: 23345593
  5. Results describe the tissue distribution of SCO1 and SCO2 in mouse and human tissues. PMID: 20864674
  6. SCO1 facilitates the transfer of copper from SCO2 to the CuA site at an early stage of COX assembly in mitochondria. PMID: 15229189
  7. data suggest that both Cu(I) and Cu(II) binding are critical for normal Sco function. PMID: 16091356
  8. Cox17-mediated copper metallation of Sco1, as well as the subsequent failure of Cu(A) site maturation, is the basis for the inefficient assembly of the cytochrome c oxidase complex in SCO1 patients PMID: 16520371
  9. Sco1 has evolved to bind a metal atom via the di-Cys motif to act as a copper chaperone, the oxidized form of the nickel-bound protein suggests that it may also maintain the thioredoxin function. PMID: 16735468
  10. These results suggest a mitochondrial pathway for the regulation of cellular copper content that involves signaling through SCO1 and SCO2, perhaps by their thiol redox or metal-binding state. PMID: 17189203
  11. Cu(I)HCox17(2S-S), i.e., the copper-loaded form of the protein, can transfer simultaneously copper(I) and two electrons to the human cochaperone Sco1 (HSco1) in the oxidized state, i.e., with its metal-binding cysteines forming a disulfide bond. PMID: 18458339
  12. a fraction of Sco1 physically associates with the cytochrome c oxidase complex in human muscle mitochondria, suggesting a possible direct relationship between CcO and the regulation of cellular copper homeostasis PMID: 19295170
  13. SCO2 acts upstream of SCO1, and that it is indispensable for CO II synthesis. PMID: 19336478

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Involvement in disease
Mitochondrial complex IV deficiency (MT-C4D)
Subcellular Location
Mitochondrion. Mitochondrion inner membrane; Single-pass membrane protein.
Protein Families
SCO1/2 family
Tissue Specificity
Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.
Database Links

HGNC: 10603

OMIM: 220110

KEGG: hsa:6341

STRING: 9606.ENSP00000255390

UniGene: Hs.14511

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