DUX4 Antibody (P4H2) [Allophycocyanin]

• Reactivity: Hu
• Applications: WB, ELISA, ICC/IF, IHC
• Clone: P4H2
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Allophycocyanin

DUX4 Antibody (P4H2) [Allophycocyanin] Summary

• Immunogen: Synthetic peptide corresponding to the C-terminus of human DUX4. [Swiss-Prot: Q9UBX2]
• Localization: Nucleus. Note: Actively transported through the nuclear pore complex (NPC)
• Specificity: This antibody is specific for DUX4 and does not recognize DUX4c.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: DUX4
• Purity: Protein G purified

Applications/Dilutions

• Dilutions:
  • ELISA
  • Immunocytochemistry/ Immunofluorescence
  • Immunohistochemistry
  • Immunohistochemistry-Frozen
  • Immunohistochemistry-Paraffin
  • Western Blot
• Application Notes: This DUX4 antibody is useful for Immunocytochemistry/Immunofluorescence, Immunohistochemistry frozen sections, Immunohistochemistry paraffin embedded sections and Western Blot. In Western blot, a band can be seen at ~52 kDa. The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
• Theoretical MW: 52 kDa.
  Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: PBS
• Preservative: 0.05% Sodium Azide
• Purity: Protein G purified

Alternate Names for DUX4 Antibody (P4H2) [Allophycocyanin]

• double homeobox 4
• double homeobox protein 10
• double homeobox protein 4
• double homeobox protein 4/10
• double homeobox protein DUX10
• DUX10
• DUX4
• DUX4L1

Background

The DUX4 gene is located within a D4Z4 repeat array in the subtelomeric region of chromosome 4q. The D4Z4 repeat is polymorphic in length; a similar D4Z4 repeat array has been identified on chromosome 10. DUX4 (also known as Double homeobox 4) is the leading candidate causative gene for facioscapulohumeral dystrophy (FSHD), a degenerative skeletal muscle disease and one of the most common muscular dystrophies. FSHD is caused by the deletion of a subset of D4Z4 macrosatellite repeats on chromosome 4. Each repeat contains a retrogene encoding the double-homeobox factor DUX4. DUX4 expression is epigenetically suppressed in differentiated tissues and the residual DUX4 transcripts are spliced to remove the carboxyterminal domain that has been associated with cell toxicity. In FSHD individuals, the expression of the full-length DUX4 transcript is not completely suppressed in skeletal muscle, and possibly other differentiated tissues, and results in a small percentage of cells expressing relatively abundant amounts of the full-length DUX4 mRNA and protein. [Snider et al. (2010) PLoS Genetics.6(10): e1001181]

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: DUX4