Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      KAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWE ISKQIEGHTICALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS
    • Molecular weight

      37 kDa including tags
    • Amino acids

      365 to 464

Specifications

Our Abpromise guarantee covers the use of ab117059 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • 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
    • NADH ubiquinone oxidoreductase 51 kDa subunit
    • NADH ubiquinone oxidoreductase core subunit V1
    • NADH-ubiquinone oxidoreductase 51 kDa subunit
    • NDUFV 1
    • ndufv1
    • NDUV1_HUMAN
    • UQOR1
    see all
  • Function

    Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
  • Involvement in disease

    Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
    Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
  • Sequence similarities

    Belongs to the complex I 51 kDa subunit family.
  • Cellular localization

    Mitochondrion inner membrane.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE showing ab117059 at approximately 36.63 kDa.
    Stained with Coomassie Blue.

References

ab117059 has not yet been referenced specifically in any publications.

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