Date published: 2025-10-18

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atrophin-1 Antibody (C1): sc-517594

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Datasheets
  • atrophin-1 Antibody (C1) is a mouse monoclonal IgG2b κ atrophin-1 antibody provided at 100 µg/ml
  • raised against recombinant atrophin-1 of human origin
  • recommended for detection of atrophin-1 of human origin by WB, IF and IHC(P)
  • At present, we have not yet completed the identification of the preferred secondary detection reagent(s) for atrophin-1 Antibody (C1). This work is in progress.

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    atrophin-1 Antibody (C1) is a mouse monoclonal IgG2b kappa light chain antibody that detects atrophin-1 protein of human origin by western blotting (WB), immunofluorescence (IF), and immunohistochemistry with paraffin-embedded sections (IHCP). anti-atrophin-1 antibody (C1) is available as a non-conjugated format. atrophin-1, also known as dentatorubral-pallidoluysian atrophy protein, plays a crucial role in neuronal function and is involved in the regulation of gene expression and cellular signaling pathways. Proper functioning of atrophin-1 is vital for maintaining neuronal health, and disruptions in atrophin-1 activity can lead to severe neurodegenerative disorders. atrophin-1 is predominantly expressed in the ovary, testis, brain, and prostate, with additional presence in the thymus, liver, and leukocytes. Notably, defects in the ATN1 gene, which encodes atrophin-1, are linked to dentatorubral-pallidoluysian atrophy (DRPLA) and Haw River syndrome (HRS), both of which are dominant neurodegenerative disorders characterized by an expansion of polyglutamine repeats. The severity and onset of these conditions correlate with the number of repeats, with affected individuals exhibiting a range of symptoms including dementia, epilepsy, and cerebellar ataxia. atrophin-1 (C1) monoclonal antibody is an essential tool for researchers studying these complex diseases and the underlying mechanisms of neuronal degeneration.

    For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.

    Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA

    LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences

    atrophin-1 Antibody (C1) References:

    1. Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.  |  Schilling, G., et al. 1999. Neuron. 24: 275-86. PMID: 10677044
    2. Protein binding of a DRPLA family through arginine-glutamic acid dipeptide repeats is enhanced by extended polyglutamine.  |  Yanagisawa, H., et al. 2000. Hum Mol Genet. 9: 1433-42. PMID: 10814707
    3. Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain.  |  Yazawa, I., et al. 1995. Nat Genet. 10: 99-103. PMID: 7647802
    4. Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA).  |  Nagafuchi, S., et al. 1994. Nat Genet. 8: 177-82. PMID: 7842016
    5. DRPLA gene (atrophin-1) sequence and mRNA expression in human brain.  |  Margolis, RL., et al. 1996. Brain Res Mol Brain Res. 36: 219-26. PMID: 8965642
    6. Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis.  |  Miyashita, T., et al. 1997. J Biol Chem. 272: 29238-42. PMID: 9361003
    7. Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins.  |  Wood, JD., et al. 1998. Mol Cell Neurosci. 11: 149-60. PMID: 9647693
    8. Dentatorubral-pallidoluysian atrophy or Naito-Oyanagi disease.  |  Kanazawa, I. 1998. Neurogenetics. 2: 1-17. PMID: 9933295

    Ordering Information

    Product NameCatalog #UNITPriceQtyFAVORITES

    atrophin-1 Antibody (C1)

    sc-517594
    100 µg/ml
    $316.00