DUX4抗体;DUX4 Antibody—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P13289(DUX4 Antibody) at dilution 1/30. (Original magnification: ×200)    

  
  

The DUX4 antibody is a critical tool in studying the role of the *DUX4* gene, which encodes a double homeobox transcription factor. Normally silenced in most somatic tissues after early embryonic development, DUX4 becomes aberrantly reactivated in facioscapulohumeral muscular dystrophy (FSHD), a debilitating muscle-wasting disorder. In FSHD, chromatin relaxation due to mutations or epigenetic changes on chromosome 4q35 leads to DUX4 expression in skeletal muscle, triggering cytotoxic gene programs that drive muscle degeneration. DUX4 antibodies are designed to detect the DUX4 protein, aiding in research to understand its pathological mechanisms, regulation, and therapeutic targeting.

These antibodies are used in techniques like Western blotting, immunofluorescence, and chromatin immunoprecipitation (ChIP) to visualize DUX4 localization, expression levels, or DNA-binding activity. However, detecting endogenous DUX4 is challenging due to its low abundance in FSHD patient samples and variable specificity of commercial antibodies. Clones such as E5-5 and 9A12 are widely cited, with E5-5 targeting the N-terminal region and 9A12 recognizing the C-terminal domain. Validation using DUX4-knockout cell lines or competitive peptide blocking is essential to confirm antibody specificity.

DUX4 antibodies also have emerging roles in cancer research, as DUX4 is ectopically expressed in certain malignancies. Despite their utility, variability in antibody performance across studies underscores the need for standardized validation protocols and improved reagents to advance FSHD and oncology research.