Product Name:
DUX4
Product Number:
ab-nn243-1
Target Full Name: Double homeobox protein 4
Target Alias: Double homeobox 4; Double homeobox 10; DUX10; Double homeobox protein 4/10
Product Type Specific: DUX4 pan-specific antibody
Antibody Code: NN243-1
Antibody Target Type: Pan-specific
Protein UniProt: Q9UBX2
Protein SigNET: DUX4
Antibody Type: Monoclonal
Antibody Host Species: Mouse
Antibody Ig Isotype Clone: IgG1
Antibody Immunogen Source: C-terminal 76 amino acids of DUX4
Production Method: Protein G purified
Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.
Antibody Concentration: 1 mg/ml
Storage Buffer: Phosphate buffered saline pH7.4, 50% glycerol, 0.09% sodium azide
Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.
Product Use: Western blotting | Immunohistochemistry | ICC/Immunofluorescence
Antibody Dilution Recommended: WB (1:1000); optimal dilutions for assays should be determined by the user.
Antibody Potency: Detects a ~45 kDa protein in cell and tissue lysates by Western blotting.
Antibody Species Reactivity: This antibody detects the target protein in the following species due to conservation of amino acid sequence: Human | Mouse.
Antibody Positive Control: 1ug/ml of SMC-192 was sufficient for detection of DUX4 in 20ug of HeLa cell lysate by ECL immunoblot analysis using goat anti-mouse IgG: HRP as teh secondary.
Antibody Cross Reactivity: No cross-reactivity with DUX4c.
Scientific Background: DUX4, or double homeobox4, is a human protein that is a transcriptional activator of paired-like homeodomain transcription factor 1 (1). Its aberrant expression in skeletal muscle causes Facioscapulohumeral Muscular Dystrophy (FSHD), where it appears to have a toxic gain of function (2-4). In FSHD individuals, the expression of the full-length DUX4 transcript is not completely suppressed in skeletal muscle and possibly other differentiated tissues (5). This acts as a toxin in skeletal muscle, causing muscle atrophy, cellular dysfunction, and disrupting RNA metabolism. DUX4 it drives expression of genes and retroelements, playing a key role in the zygotic genome activation during early human development. It is normally expressed in the 4-cell embryo in the 4-cell embryo, germline cells (testis), and some immune-privileged sites. DUX4 expression is linked to activating E3 ligases, potential protein aggregation (e.g., TDP-43), and suppression of apoptosis in certain contexts, ultimately leading to myocyte death. Its pathological upregulation in muscle tissue leads to the deregulation of hundreds of genes. In addition to high expression in FSHD muscle cells, it is found in a high percentage of various cancer types (e.g., specific B-ALL subtypes). This description may include information annotated by UniProt and/or Google AI.