DUX4/DUX4c Antibody (2143C) [Unconjugated]

Western blot shows lysates of C2C12 mouse myoblast cell line transfected with human DUX4 untreated (-) or treated (+) with 100 ng/mL Doxycycline for overnight. PVDF membrane was probed with 1 µg/mL of Rabbit Anti-Human DUX4 Monoclonal Antibody (Catalog # MAB95351) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # HAF008). A specific band was detected for DUX4 at approximately 55 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

• Reactivity: Hu
• Applications: WB
• Clone: 2143C
• Clonality: Monoclonal
• Host: Rabbit
• Conjugate: Unconjugated

DUX4/DUX4c Antibody (2143C) [Unconjugated] Summary

• Additional Information: Recombinant Monoclonal Antibody.
• Immunogen: Human DUX4 synthetic peptide containing sequences within amino acids 200-300 of the human DUX4 protein
  Accession # Q9UBX2
• Specificity: Detects human DUX4/DUX4c in direct ELISAs.
• Source: N/A
• Isotype: IgG
• Clonality: Monoclonal
• Host: Rabbit
• Purity Statement: Protein A or G purified from cell culture supernatant

Applications/Dilutions

• Dilutions:
  • Western Blot 1 ug/mL

Packaging, Storage & Formulations

• Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
• Buffer: Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
• Reconstitution Instructions: Reconstitute at 0.5 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for DUX4/DUX4c Antibody (2143C) [Unconjugated]

• DUX10
• DUX4
• DUX4/DUX4c
• DUX4L

Background

Human Double homeobox 4 (aka DUX4) is a protein encoded by the DUX4 gene. This gene is located within a D4Z4 repeat array in chromosome 4q35. Each D4Z4 repeat unit has an open reading frame, DUX4, that contains two homeoboxes. Unregulated expression of DUX4 in muscle cells is the cause of facioscapulohumeral muscular dystrophy (FSHD), a common form of muscular dystrophy in adults that is one of the most prevalent genetic diseases of muscle.

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.

Publications for DUX4/DUX4c Antibody (MAB95351)(5)

Publications using MAB95351

• Campolungo, D;Salomé, M;Biferali, B;Tascini, AS;Gabellini, D; DUX4-r exerts a neomorphic activity that depends on GTF2I in acute lymphoblastic leukemia Science advances 2023-09-15 [PMID: 37713484] (Western Blot, Human)
• HAN Soliman, EA Toso, IE Darwish, SM Ali, M Kyba Antiapoptotic Protein FAIM2 is targeted by miR-3202, and DUX4 via TRIM21, leading to cell death and defective myogenesis Cell Death & Disease, 2022-04-25;13(4):405. 2022-04-25 [PMID: 35468884] (Western Blot, Human)
• D Bosnakovsk, D Oyler, A Mitanoska, M Douglas, ET Ener, AS Shams, M Kyba Persistent Fibroadipogenic Progenitor Expansion Following Transient DUX4 Expression Provokes a Profibrotic State in a Mouse Model for FSHD International Journal of Molecular Sciences, 2022-02-11;23(4):. 2022-02-11 [PMID: 35216102] (IHC, Mouse)
• D Bosnakovsk, AS Shams, C Yuan, MT da Silva, ET Ener, CW Baumann, AJ Lindsay, M Verma, A Asakura, DA Lowe, M Kyba Transcriptional and cytopathological hallmarks of FSHD in chronic DUX4-expressing mice J. Clin. Invest., 2020-05-01;0(0):. 2020-05-01 [PMID: 32250341] (IHC, Mouse)
• JK Lee, D Bosnakovsk, EA Toso, T Dinh, S Banerjee, TE Bohl, K Shi, K Orellana, M Kyba, H Aihara Crystal Structure of the Double Homeodomain of DUX4 in Complex with DNA Cell Rep, 2018-12-11;25(11):2955-2962.e3. 2018-12-11 [PMID: 30540931] (Western Blot, Mouse)

Bioinformatics

• Uniprot:
  • Q9UBX2()