Human SCO2 Recombinant Protein (RPPB4598)
- SKU:
- RPPB4598
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- O43819
Description
| Product Name: | Human SCO2 Recombinant Protein |
| Product Code: | RPPB4598 |
| Size: | 10µg |
| Species: | Human |
| Target: | SCO2 |
| Synonyms: | SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | The SCO2 protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 200mM NaCl and 30% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks.�Store, frozen at -20°C for longer periods of time.Please avoid freeze thaw cycles. |
| Purity: | Greater than 90% as determined by SDS-PAGE. |
| 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 |
SCO2 protein is a member of the SCO1/2 family. SCO1 and SCO2 proteins are found on the inner membrane of the mitochondria and takes a vital part copper insertion or transport to the active site of cytochrome c oxidase (COX). Flaws in SCO2 are the reason for deadly infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) which is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle display declines in cytochrome c oxidase (COX) activity, while liver and fibroblasts show mild COX deficiencies.
SCO2 produced in E.Coli is a single, non-glycosylated polypeptide chain containing 246 amino acids (42-266a.a.) and having a molecular mass of 27.4kDa.SCO2 is fused to a 21 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | SCO2: Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2). 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. Belongs to the SCO1/2 family. |
| UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 22q13.33 Cellular Component: nucleoplasm; myofibril; mitochondrion; mitochondrial inner membrane Molecular Function:copper ion binding Biological Process: cellular copper ion homeostasis; eye development; in utero embryonic development; respiratory chain complex IV assembly; response to activity; muscle system process; copper ion transport Disease: Cardioencephalomyopathy, Fatal Infantile, Due To Cytochrome C Oxidase Deficiency 1; Myopia 6 |
| NCBI Summary: | 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 Code: | O43819 |
| NCBI GenInfo Identifier: | 8134662 |
| NCBI Gene ID: | 9997 |
| NCBI Accession: | O43819.3 |
| UniProt Secondary Accession: | O43819,Q3T1B5, Q9UK87, |
| UniProt Related Accession: | O43819 |
| Molecular Weight: | 29,810 Da |
| NCBI Full Name: | Protein SCO2 homolog, mitochondrial |
| NCBI Synonym Full Names: | SCO2 cytochrome c oxidase assembly protein |
| NCBI Official Symbol: | SCO2�� |
| NCBI Official Synonym Symbols: | MYP6; SCO1L; CEMCOX1�� |
| NCBI Protein Information: | protein SCO2 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 2 |
| UniProt Protein Name: | Protein SCO2 homolog, mitochondrial |
| Protein Family: | SCO2-like protein |
| UniProt Gene Name: | SCO2�� |
| UniProt Entry Name: | SCO2_HUMAN |
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