Human SCO1 Recombinant Protein (RPPB4597)
- SKU:
- RPPB4597
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- O75880
Description
| Product Name: | Human SCO1 Recombinant Protein |
| Product Code: | RPPB4597 |
| Size: | 20µg |
| Species: | Human |
| Target: | SCO1 |
| Synonyms: | SCO1 Cytochrome C Oxidase Assembly Protein, SCOD1, SCO(Cytochrome Oxidase Deficient, Yeast) Homolog 1, SCO Cytochrome OxidaseDeficient Homolog 1 (Yeast), SCO Cytochrome Oxidase Deficient Homolog 1, ProteinSCO1 Homolog, Mitochondrial, SCOD1, Protein SCO1 homolog, mitochondrial. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | SCO1 protein solution (1mg/ml) containing Phosphatebuffered saline (pH7.4), 10% glycerol and 1mM DTT. |
| 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. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 95% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGKPLLGGPF SLTTHTGERK TDKDYLGQWL LIYFGFTHCP DVCPEELEKMIQVVDEIDSI TTLPDLTPLF ISIDPERDTK EAIANYVKEF SPKLVGLTGT REEVDQVARA YRVYYSPGPKDEDEDYIVDH TIIMYLIGPD GEFLDYFGQN KRKGEIAASI ATHMRPYRKK SLEHHHHHH |
SCO Cytochrome Oxidase Deficient Homolog 1, also known as SCO1 is amember of the SCO1/2 family. Mammalian cytochrome c oxidase (COX) catalyzes thetransfer of reducing equivalents from cytochrome c to molecular oxygen andpumps protons across the inner mitochondrial membrane. Furthermore, in yeast, tworelated COX assembly genes, SCO1 & SCO2 which are synthesis of cytochrome coxidase, enable subunits 1 as well as 2 to be incorporated into theholoprotein. This gene is the human homolog to the yeast SCO1 gene. Among the diseasesassociated with SCO1 are hepatic failure, early-onset, neurologic disorder dueto cytochrome c oxidase deficiency and fatal infantile cytochrome c oxidasedeficiency.
SCO1 Human Recombinant produced in E.Coli is a single,non-glycosylated polypeptide chain containing 179 amino acids (132-301 a.a) andhaving a molecular mass of 20.5kDa.SCO1 is fused to a 9 amino acid His-tag at C-terminus& purified by proprietary chromatographic techniques.
| UniProt Protein Function: | SCO1: Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX. Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D); also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Belongs to the SCO1/2 family. |
| UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 17p13.1 Cellular Component: mitochondrion; myofibril; mitochondrial inner membrane Molecular Function:copper ion binding Biological Process: generation of precursor metabolites and energy; cellular copper ion homeostasis; respiratory chain complex IV assembly; copper ion transport Disease: Mitochondrial Complex Iv Deficiency |
| NCBI Summary: | Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog to the yeast SCO1 gene. [provided by RefSeq, Jul 2008] |
| UniProt Code: | O75880 |
| NCBI GenInfo Identifier: | 8134663 |
| NCBI Gene ID: | 6341 |
| NCBI Accession: | O75880.1 |
| UniProt Secondary Accession: | O75880,B2RDM0, |
| UniProt Related Accession: | O75880 |
| Molecular Weight: | 33,814 Da |
| NCBI Full Name: | Protein SCO1 homolog, mitochondrial |
| NCBI Synonym Full Names: | SCO1 cytochrome c oxidase assembly protein |
| NCBI Official Symbol: | SCO1�� |
| NCBI Official Synonym Symbols: | SCOD1�� |
| NCBI Protein Information: | protein SCO1 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 1 |
| UniProt Protein Name: | Protein SCO1 homolog, mitochondrial |
| Protein Family: | SCO1 protein |
| UniProt Gene Name: | SCO1�� |
| UniProt Entry Name: | SCO1_HUMAN |
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