SCO2

Alternative Names: SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.

The SCO2 protein, a member of the SCO1/2 family, is located on the inner membrane of mitochondria and plays a crucial role in the insertion or transport of copper to the active site of cytochrome c oxidase (COX). Defects in SCO2 result in a severe condition called fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC), which is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. The heart and skeletal muscles exhibit decreased COX activity, while the liver and fibroblasts display mild deficiencies in COX.

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  • Sku: PSB-pro-054-2µg
  • Vendor: ProSpec-Tany
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Safety Data Sheet
UniProt Listing
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Product Details

Product Description

Recombinant Human SCO Cytochrome Oxidase Deficient Homolog 2, also known as SCO2, is a protein that belongs to the SCO2 family. This protein plays a crucial role in the assembly of cytochrome c oxidase, a key enzyme involved in cellular respiration. With a molecular weight of approximately X kDa, SCO2 is involved in the regulation of mitochondrial function and energy production. Understanding the biochemical properties and functions of SCO2 can provide insights into mitochondrial disorders and potential therapeutic interventions.

Current Lead Time

7-14 Days

Product Specifications

Species
 Human
Expression System E. coli
Purity 90% by SDS-PAGE
Activity
 Not Available
Activity Assay
Animal Component Free (ACF) Yes
Molecular Weight
 27.4 kDa
Structure Homodimer
Endotoxin Concentration
 Not Available
Purification Method Not Available
Form
 Frozen
Formulation The SCO2 protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 200mM NaCl and 30% glycerol.

*For Research Use Only

Product Guarantee

If your product is within its expiration period, and has not performed according to expectations, fill out the sample return form. Return the unused sample frozen, with the auto generated packing slip to the address on the instructions. You will receive a full refund along with a replacement of the unused portion (inclusive of the failed test). Damages do not cover any additional costs incurred for failed experimentation. 

Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MGPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHR GRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ ASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS

Storage Instruction

Reconstituted store at -20°C or -80°C.We recommend that single use aliquots are prepared to avoid freeze-thaw cycles.

Sample Prep Instruction

1. Thaw the vial on ice, spin briefly
2. Prepare single-use or stock aliquots. This stage will depend on your final application so please adapt as appropriate. All our proteins are supplied carrier protein-free. If compatible with your work and you are storing at lower concentrations (<50 ug/ml) adding carrier protein is highly advised (usually 1% w/v high purity BSA or equivalent – ensure all buffers are sterile-filtered).
3. Prepare single-use aliquots whenever possible to avoid freeze-thaw cycles which can damage the proteins and reduce bioactivity. Store aliquots at -70 °C (or -20 °C)
4. We recommend sterile filtering after dilution in media or the final working solution
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References

Not Available
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SCO2
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