Recombinant Human NDUFV1 protein (ab117059)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP49821
    • SpeciesHuman
    • SequenceKAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWE ISKQIEGHTICALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS
    • Molecular weight37 kDa including tags
    • Amino acids365 to 464

Associated products

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

  • FormLiquid
  • Additional notesProtein 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
    • 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 51 kDa subunit
    • NDUFV 1
    • ndufv1
    • NDUV1_HUMAN
    • UQOR1
    see all
  • FunctionCore 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 diseaseDefects 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 similaritiesBelongs to the complex I 51 kDa subunit family.
  • Cellular localizationMitochondrion inner membrane.
  • Information by UniProt

Recombinant Human NDUFV1 protein images

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

References for Recombinant Human NDUFV1 protein (ab117059)

ab117059 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab117059.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"