Product Name
SCO2, Recombinant Protein
Full Product Name
SCO2, 42-266aa, Human, His tag, E Coli
Product Synonym Names
Protein SCO2 homolog mitochondrial; Protein SCO2 homolog mitochondrial; SCO1L
Product Gene Name
SCO2 recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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
OMIM
AF177385 Genomic DNA
3D Structure
ModBase 3D Structure for O43819
Purity/Purification
> 90% by SDS-PAGE
Form/Format
Liquid. 20mM Tris-HCl Buffer (pH 8.0) containing 30% glycerol, 2mM DTT, 200mM NaCl
Concentration
0.5 mg/ml (determined by Bradford assay) (lot specific)
Preparation and Storage
Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.
Other Notes
Small volumes of SCO2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
SCO2 recombinant protein
SCO2 protein belongs to the SCO1/2 family of proteins. Both SCO1 and SCO2 proteins are located on the inner membrane of the mitochondria and plays a crucial role in copper insertion or transport to the active site of cytochrome c oxidase (COX). Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC). This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies. Recombinant human SCO2 protein, fused to His-tag at N-terminus, was expressed in E Coli and purified by using conventional chromatography techniques.
Product Categories/Family for SCO2 recombinant protein
Metabolism
Applications Tested/Suitable for SCO2 recombinant protein
SDS-PAGE
SDS-Page of SCO2 recombinant protein
NCBI/Uniprot data below describe general gene information for SCO2. It may not necessarily be applicable to this product.
NCBI Accession #
NP_005129
[Other Products]
NCBI GenBank Nucleotide #
NM_005138.2
[Other Products]
UniProt Primary Accession #
O43819
[Other Products]
UniProt Secondary Accession #
Q3T1B5; Q9UK87[Other Products]
UniProt Related Accession #
O43819[Other Products]
Molecular Weight
27.4kDa (246aa), confirmed by MALDI-TOF
NCBI Official Full Name
protein SCO2 homolog, mitochondrial
NCBI Official Synonym Full Names
SCO2, cytochrome c oxidase assembly protein
NCBI Official Symbol
SCO2??[Similar Products]
NCBI Official Synonym Symbols
MYP6; SCO1L; CEMCOX1
??[Similar Products]
NCBI Protein Information
protein SCO2 homolog, mitochondrial
UniProt Protein Name
Protein SCO2 homolog, mitochondrial
Protein Family
SCO2-like protein
UniProt Gene Name
SCO2??[Similar Products]
NCBI Summary for SCO2
Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors; this gene encodes one of the COX assembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy and myopia 6. [provided by RefSeq, Oct 2014]
UniProt Comments for SCO2
Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).
Product References and Citations for SCO2 recombinant protein
Meister G., et al. (2001) EMBO J. 20:2304-2314; Talbot K., et al. (1998) Hum. Mol. Genet. 7:2149-2156;
Research Articles on SCO2
1. We identified one novel possibility of an extreme myopia-causing mutation in SCO2. No other disease-causing mutation was found in 101 extremely myopic Japanese patients, suggesting that SCO2 plays a limited role in Japanese extreme myopia.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
