Desmin polyclonal, anti-human, mouse, rat

Background:
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.

Synonyms: CSM1, CSM2, LGMD2R, DES, Desmin

Cellular Location: Cell membrane Cytoplasm sarcolemma

Immunogen:
A synthetic peptide of human Desmin

Gene Symbol: Desmin

Gene ID: 1674

Swiss prot: P17661

Calculated MW: 65kDa

Recommended dilution:
WB 1:3000

Purity:
Affinity purification

Storage Buffer:
Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3.

Storage:
Store at -20℃. Avoid freeze / thaw cycles.