DATASHEET
Host:
Rabbit
Target Protein:
Desmin Thr76 + Thr77
Modification Site:
Thr76 + Thr77
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
1674
Source:
KLH conjugated synthetic phosphopeptide derived from human DES around the phosphorylation site of Thr76/Thr77
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4°C. Store at -20°C for one year. 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.