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Recombinant human SCO2 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 Specifications

Product Name Alternative

Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1L

Expression System

E.coli

Antigen Species

Human

Tag

His-Tag

Applications

SDS-PAGE

Concentration

0.5 mg/mL (determined by Bradford assay)

Purity

> 90% by SDS-PAGE

Molecular Weight

27.4 kDa (246aa) confirmed by MALDI-TOF

Additionnal Information

SCO2, Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1L, ATGP0867-10 µg, ATGP0867-20 µg, ATGP0867-50 µg, ATGP0867-100 µg, ATGP0867-250 µg, ATGP0867-500 µg, ATGP0867-1 mg, ATGP0867-10, ATGP0867-20, ATGP0867-50, ATGP0867-100, ATGP0867-250, ATGP0867-500, ATGP0867-1

References & Citations

Meister G., et al. (2001) EMBO J. 20:2304-2314; ; Talbot K., et al. (1998) Hum. Mol. Genet. 7:2149-2156;

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 30% glycerol, 2mM DTT, 200 mM NaCl

Scientific Category

Metabolism

NCBI Accession Number

NP_005129

Uniprot Accession Number

O43819

Species

Human

AA 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

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