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Recombinant Human NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (NDUFV1)

ACP23194

Number
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High Purity LevelsPrecision and ReliabilityCustomization Options

Specifications


Cat.No ACP23194 Target NameNDUFV1
FormLyophilized powderExpression SystemCustom Production. Please inquire and provide the desire expression system.
Expression Range21-464Protein LengthFull Length of Mature Protein
Purity>85% (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 IDP49821
Background Information
  • Uniprot Id

    P49821

  • Target Species

    Human

  • Target Name

    NDUFV1

  • Target Full Name

    NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial

  • Target Function

    Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor.

  • Target Involvement

    Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)

  • Target Subcellular Location

    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

  • Target Protein Families

    Complex I 51 kDa subunit family

  • Target Synonyms

    CI 51kD; CI-51kD; CI51KD; Complex I 51kD; Complex I-51kD; FLJ59059; mitochondrial; NADH dehydrogenase (ubiquinone) flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; NADH dehydrogenase flavoprotein 1; NADH ubiquinone oxidoreductase 51 kDa subunit; NADH ubiquinone oxidoreductase; NADH ubiquinone oxidoreductase core subunit V1; NADH-ubiquinone oxidoreductase 51 kDa subunit; NDUFV 1; ndufv1; NDUV1_HUMAN; UQOR1

  • Target Background

    The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10, 000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

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