Overview

  • Product name

  • Description

    Rabbit polyclonal to NDUFV1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human NDUFV1 aa 50-100 conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary.
    Sequence:

    DWRLKGSLSRGDWYKTKEILLKGPDWILGEIKTSGLRGRGGAGFPTGLKW S


    Database link: P49821

  • Positive control

    • Rat brain tissue

Properties

Applications

Our Abpromise guarantee covers the use of ab203208 in the following tested applications.

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

Application Abreviews Notes
IHC-P 1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Use at 1/50 - 1/200 with fluorescent detection methods.

WB 1/100 - 1/1000. Predicted molecular weight: 51 kDa.

Target

  • 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
  • Database links

  • Alternative names

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

Images

  • Immunohistochemical analysis of formalin-fixed and paraffin embedded Rat brain tissue labeling NDUFV1 using ab203208 at 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.

References

ab203208 has not yet been referenced specifically in any publications.

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