Overview

  • Product name
    Anti-NDUFV1 antibody - N-terminal
    See all NDUFV1 primary antibodies
  • Description
    Rabbit polyclonal to NDUFV1 - N-terminal
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Cow, Cynomolgus monkey
  • Immunogen

    Synthetic peptide within Human NDUFV1 aa 20-50 (N terminal) conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary.
    Database link: P49821

  • Positive control
    • MDA-MB453 cell lysate.

Applications

Our Abpromise guarantee covers the use of ab174472 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
WB 1/100 - 1/500. 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

  • Anti-NDUFV1 antibody - N-terminal (ab174472) at 1/100 dilution + MDA-MB453 cell lysate at 35 µg

    Predicted band size : 51 kDa

References

This product has been referenced in:
  • Nafisinia M  et al. Whole Exome Sequencing Identifies the Genetic Basis of Late-Onset Leigh Syndrome in a Patient with MRI but Little Biochemical Evidence of a Mitochondrial Disorder. JIMD Rep 32:117-124 (2017). WB ; Human . Read more (PubMed: 27344648) »

See 1 Publication for this product

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

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