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SCO1 Human

SCO Cytochrome Oxidase Deficient Homolog 1 Human Recombinant

 
Catalog #
RCP2003
Uniprot Id
O75880
 
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 Catalog #AvailabilitySizeQuantityUnit Price Save For Later Wish List
RCP2003-5 7 days 5 µg $120.00
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RCP2003-20 7 days 20 µg $290.00
RCP2003-1 7 days 1 mg $4,320.00
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Product Overview

NameSCO1 Human
Description
SCO Cytochrome Oxidase Deficient Homolog 1 Human Recombinant
Accession (Primary)O75880
Synonyms
SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.
Introduction
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.
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
Precautions
SCO1 Human is for research use only and not for use in diagnostic or therapeutic procedures.

Target Information: ( O75880 )

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