bs-20702R-Biotin

DATASHEET

Host: Rabbit

Target Protein: Desmin

Immunogen Range: 311-400/470

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 1674

Swiss Prot: P17661

Source: KLH conjugated synthetic peptide derived from human Desmin

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 for 12 months.

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 antibodies are useful in identification of tumours of myogenic origin.

Conjugation: Biotin

Excitation/ Emission: N/A

Size: 100µL

Concentration: 1ug/ul

Applications: IHC-P(1:200-400)
IHC-F(1:100-500)
IF(ICC)(1:50-200)
IF(1:50-200)

Predicted Molecular Weight: 52 kDa

Cross Reactive Species: Rat

Predicted Cross Reactive Species: Human
Mouse
Dog
Cow
Sheep
Pig
Horse
Rabbit

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