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Recombinant Human Protein SCO2 homolog, mitochondrial (SCO2)

ACP04020

Number
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High Purity LevelsPrecision and ReliabilityCustomization Options

Specifications


Cat.No ACP04020 Target NameSCO2
FormLiquid or Lyophilized powderExpression SystemE.coli
Expression Range43-266aaMol Weight41.1kDa
Protein LengthFull Length of Mature ProteinPurityGreater than 90% as determined by SDS-PAGE.
Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDO43819
Background Information
  • Uniprot Id

    O43819

  • Target Species

    Human

  • Target Name

    SCO2

  • Target Full Name

    Protein SCO2 homolog, mitochondrial

  • Target Function

    Copper metallochaperone essential for the synthesis and maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Involved in transporting copper to the Cu(A) site on MT-CO2/COX2. Also acts as a thiol-disulfide oxidoreductase to regulate the redox state of the cysteines in SCO1 during maturation of MT-CO2/COX2.

  • Target Involvement

    Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 1 (CEMCOX1); Myopia 6 (MYP6); Leigh syndrome (LS)

  • Target Subcellular Location

    Mitochondrion inner membrane; Single-pass membrane protein.

  • Target Protein Families

    SCO1/2 family

  • Target Tissue Specificity

    Ubiquitous.

  • Target Research Area

    Metabolism

  • Target Synonyms

    Cytochrome oxidase deficient homolog 2; MGC125823; MGC125825; OTTHUMP00000196774; OTTHUMP00000196775; Protein SCO2 homolog; mitochondrial; SCO (cytochrome oxidase deficient; yeast) homolog 2; SCO 1L; SCO 2; SCO cytochrome oxidase deficient homolog 2 (yeast); SCO cytochrome oxidase deficient homolog 2; SCO1L; SCO2; SCO2_HUMAN; Synthesis of cytochrome c oxidase 2

  • Target Background

    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.

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