Human SCO1 Recombinant Protein (RPPB4597)

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SKU:
RPPB4597
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
O75880

Description

system_update_altDatasheetsystem_update_altMSDS
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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system_update_altDatasheetHuman SCO1/SCOD1 Recombinant Protein Protein SCO1 Homolog, Mitochondrial (SCO1) is a member of the SCO1/2 family. SCO1 has a homodimer structure. SCO1 is located in mito