DUX4 Antibody (P4H2) - BSA Free

Simple Western: DUX4 Antibody (P4H2) [NBP1-49552] - Lane view shows a specific band for DUX4 in 0.5 mg/ml of HeLa lysate. This experiment was performed under reducing conditions using the 12-230 kDa separation system.

Immunocytochemistry/Immunofluorescence: DUX4 Antibody (P4H2) [NBP1-49552] - Detection of human DUX4 on C2C12 mouse myoblasts transfected with pCS2-DUX4. Cells were counterstained with DAPI for nuclei.

Western Blot: DUX4 Antibody (P4H2) [NBP1-49552] - Total protein from human testis tissue and human K562, HeLa and HepG2 cell lines was separated on a 12% gel by SDS-PAGE, transferred to PVDF membrane and blocked in 5% non-fat milk in TBST. The membrane was probed with 2.0 ug/ml anti-DUX4 in 1% non-fat milk in TBST and detected with an anti-mouse HRP secondary antibody using chemiluminescence.

Immunocytochemistry/Immunofluorescence: DUX4 Antibody (P4H2) [NBP1-49552] - A small number of FSHD muscle cells express a relatively large amount of DUX4. Cultured FSHD muscle cells were differentiated and immunostained with mAbs to DUX4. Cells were co-stained with the E5-5 rabbit mAb to the DUX4 C-terminal region (panels a, c, e) and the P2G4 mouse mAb to the N-term. region (panels b,d,f), or co-stained with the P4H2 mouse mAb to the C-terminal region and the E14-3 rabbit mAb to the N-term. region. Approximately 1 cell per 1000 showed nuclear staining and the co-localization of both the N-term. and C-term. regions indicates that these cells are expressing the full-length DUX4 protein. No positive nuclei were apparent in the control muscle cultures. Image collected and cropped by CiteAb from the following publication (//dx.plos.org/10.1371/journal.pgen.1001181), licensed under a CC-BY license.

Immunohistochemistry: DUX4 Antibody (P4H2) [NBP1-49552] - Analysis of DUX4 on human testis

Western Blot: DUX4 Antibody (P4H2) [NBP1-49552] - Analysis of DUX4 expression in (1) human testis and (2) DUX4 protein lysates.

• Reactivity: Hu
• Applications: WB, Simple Western, ELISA, ICC/IF, IHC
• Clone: P4H2
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: BSA Free
• Concentration: 1 mg/ml

DUX4 Antibody (P4H2) - BSA Free 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 reported in scientific literature (PMID 21529284)
  • Immunocytochemistry/ Immunofluorescence 1:100-1:200
  • Immunohistochemistry 1:100-1:200
  • Immunohistochemistry-Frozen 1:100-1:200
  • Immunohistochemistry-Paraffin 1:100-1:200
  • Simple Western 1:10
  • Western Blot 0.5-2.0ug/ml
• Application Notes: In Western blot, a band can be seen at approx. 52 kDa. In Simple Western only 10 - 15 uL of the recommended dilution is used per data point.
  See Simple Western Antibody Database for Simple Western validation: Tested in HeLa lysate 0.5 mg/mL, separated by Size, antibody dilution of 1:10, apparent MW was 61 kDa. Separated by Size-Wes, Sally Sue/Peggy Sue.
• 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 short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: PBS
• Preservative: 0.02% Sodium Azide
• Concentration: 1 mg/ml
• Purity: Protein G purified

Alternate Names for DUX4 Antibody (P4H2) - BSA Free

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

Background

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.

Publications for DUX4 Antibody (NBP1-49552)(6)

Publications using NBP1-49552

• Ganassi M, Figeac N, Reynaud M et al. Antagonism Between DUX4 and DUX4c Highlights a Pathomechanism Operating Through ?-Catenin in Facioscapulohumeral Muscular Dystrophy Frontiers in Cell and Developmental Biology 2022-09-07 [PMID: 36158201] (Western Blot, Immunocytochemistry/ Immunofluorescence, Human)
• Rashnonejad A, Chermahini G, Taylor N et al. Designed U7 small nuclear RNA antisense expression cassettes inhibit DUX4 expression and improve FSHD-associated outcomes in DUX4 over-expressing cells and FSHD patient myotubes Molecular Therapy - Nucleic Acids 2020-12-01 [PMID: 33510937] (WB, Human)
• Narayanaswamy PB, Baral TK, Haller H et al. Transcriptomic pathway analysis of urokinase receptor silenced breast cancer cells: a microarray study. Oncotarget 2017-11-24 [PMID: 29254187] (WB, Human)
• Choi IY, Lim H, Estrellas K, et al. Concordant but Varied Phenotypes among Duchenne Muscular Dystrophy Patient-Specific Myoblasts Derived using a Human iPSC-Based Model. Cell Rep. 2016-06-07 [PMID: 27239027]
• Geng LN, Tyler AE, Tapscott SJ. Immunodetection of human double homeobox 4. Hybridoma (Larchmt);30(2):125-30. 2011-04-01 [PMID: 21529284] (ICC/IF, ELISA, WB, Human)
• Snider L, Geng LN, Lemmers RJ et al. Facioscapulohumeral dystrophy: incomplete suppression of a retrotransposed gene. PLoS Genet 2010-10-01 [PMID: 21060811] (ICC/IF, WB, Human)

Bioinformatics

• Gene Symbol: DUX4
• Entrez:
  • 22947(Human)
• Uniprot:
  • Q9UBX2()