bs-12369R-FITC

DATASHEET

Host: Rabbit

Target Protein: DUX4

Immunogen Range: 53-120/424

Clonality: Polyclonal

Isotype: IgG

Source: KLH conjugated synthetic peptide derived from human DUX4

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Background:

DUX4 is a homeodomain protein with a similar protein sequence to Pax3 and Pax7. Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD). FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion.

Conjugation: FITC

Excitation/ Emission: 494nm/518nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 45

Predicted Cross Reactive Species: Human

For research use only. Not intended for diagnostic or therapeutic use.