bs-5301R-FITC

DATASHEET

Host: Rabbit

Target Protein: Desmin Thr17

Modification Site: Thr17

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 1674

Source: KLH conjugated synthetic phosphopeptide derived from human DES around the phosphorylation site of Thr17

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:

filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin are useful in identification of tumours of myogenic origin.

Conjugation: FITC

Excitation/ Emission: 494nm/518nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)

Predicted Molecular Weight: 52

Cross Reactive Species: Human
Mouse
Rat

Predicted Cross Reactive Species: Cow
Chicken

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