Recombinant humaan SCO2-eiwit

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.

Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1L

E.coli

Human

His-Tag

SDS-PAGE

0.5 mg/mL (determined by Bradford assay)

> 90% by SDS-PAGE

27.4 kDa (246aa) confirmed by MALDI-TOF

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

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

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.

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

Metabolism

NP_005129

O43819

Human

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