MilliporeSigma™ DUX4, Mouse, Unlabeled, Clone: P4H2,
Manufacturer: MilliporeSigma™
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | MABS1348-Each-of-1 | In Stock | ₹ 41,813.98 |
MABS1348 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 41,813.98
GST (18%): ₹ 7,526.516
Total Price: ₹ 49,340.496
Antigen
DUX4
Classification
Monoclonal
Concentration
Please refer to lot specific datasheet.
Formulation
Purified mouse IgG1 in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% Sodium Azide.
Gene Accession No.
Q9UBX2
Immunogen
GST-tagged recombinant human DUX4 C-terminal fragment.
Quantity
100 μg
Research Discipline
Signaling
Gene ID (Entrez)
NP_001292997
Target Species
Human
Form
Purified
Applications
Western Blot
Clone
P4H2
Conjugate
Unconjugated
Gene
DUX4, DUX10
Host Species
Mouse
Purification Method
Protein G Purified
Regulatory Status
RUO
Primary or Secondary
Primary
Test Specificity
Clone P4H2 is specific to DUX4 (UniProt Q9UBX2) and does not recognize DUX4c (DUX4L9; UniProt Q6RFH8) or the spliced DUX4 short isoform DUX4-s (UniProt E2JJS1) that lacks the C-terminal epitope region targeted by clone P4H2 (Geng, L.N., et al. (2011). Hybridoma (Larchmt). 30(2):125-130; Snider, L., et al. (2010). PLoS Genet. 6(10):e1001181).
Content And Storage
Stable for 1 year at 2°-8°C from date of receipt.
Isotype
IgG1 κ
Related Products
Description
- Double homeobox protein 4 (UniProt Q9UBX2; also known as Double homeobox protein 10) is encoded by the DUX4 (also known as DUX10) gene (Gene ID 100288687) in human
- DUX4 is a double homeobox transcription factor that is normally expressed in the testis
- Contraction or hypomethylation of D4Z4 macrosatellite repeats on chromosome 4q35 causes aberrant DUX4 expression in skeletal muscle, a hallmark of the incurable disease facioscapulohumeral muscular dystrophy (FSHD), characterized by skeletal muscle weakness and wasting
- Gene expression data analysis identifies β-catenin as the main coordinator of FSHD-associated perturbation of protein interaction signallings, including those of canonical Wnt, HIF1-α and TNF-α
- In addition, DUX4 expression causes proteolytic degradation of UPF1, a central component of the nonsense-mediated decay (NMD) machinery, and accumulation NMD substrate RNAs including DUX4 mRNA, such that NMD downregulation by DUX4 expression stabilizes DUX4 mRNA through a double-negative feedback loop in FSHD muscle cells
- FSHD muscle expresses a low abundance DUX4 mRNA that produces the full-length DUX4 protein
- In contrast to control skeletal muscle and most other somatic tissues, full-length DUX4 transcript and protein is expressed at relatively abundant levels in human testis, most likely in the germ-line cells
- Induced pluripotent (iPS) cells also express full-length DUX4 and differentiation of iPS cells to embryoid bodies suppresses expression of full-length DUX4, whereas expression of full-length DUX4 persists in differentiated FSHD iPS cells.