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DUX4 Rabbit anti-Human, Polyclonal, Bioss
SDP

Catalog No. 501982181
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100 μL
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50-198-2181 100 μL
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Catalog No. 50-198-2181 Supplier Bioss Supplier No. BS12369R
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Rabbit Polyclonal Antibody

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.

Specifications

Antigen DUX4
Applications Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 1 μg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Dux4
Gene Accession No. Q9UBX2
Gene Alias Double homeobox 10; double homeobox 4; double homeobox 4-like; Double homeobox protein 10; Double homeobox protein 4; double homeobox protein 4/10; DUX10; DUX4; DUX4 (HGNC:50800); DUX4L; DUX4L1
Gene Symbols Dux4
Host Species Rabbit
Immunogen KLH conjugated synthetic peptide derived from human DUX4.
Purification Method Protein A
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 100288687
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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