This antibody reacts specifically with Desmin (Thr76 + Thr77)
KLH conjugated synthetic phosphopeptide derived from human DES around the phosphorylation site of Thr76/Thr77
Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided.
Desmins or DES proteins detect by anti-desmin antibodies and DES ELISA kits. DSM1 and DSM2 play an important role in Desminopathy. Desmin regulated Myofibrilate Myopathy is a protein filament mutation of muscle proteins.
Cross Reactive Species details
No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.
Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.
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.