A synthetic peptide from c-terminal region of mouse Desmin conjugated to an immunogenic carrier protein was used as the immunogen. The antigen is homologous in human and rat.
Function: Desmin are class-III intermediate 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 DES 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 DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome).
Subcellular location: Cytoplasm.
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
12 months after reconstitution
This item will be shipped to you at ambient temperature in a lyophilised form.
1. Li Z, et al. Gene 78:243-254(1989)
2. Goldfarb L.G, et al. Nat. Genet. 19:402-403(1998)
3. Goudeau B, et al. Hum. Mutat. 18:388-396(2001)
4. Li H, et al. J. Cell Biol. 124:827-841(1994)
5. van Groningen J.J.M, et al. Biochim. Biophys. Acta 1217:107-109(1994)
For research use only
NZ white rabbit
Polyclonal, whole serum
IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specific for Desmin.
Mouse, human, rat. Other species not yet tested.
Reconstitute in 150 µl of sterile water. Centrifuge to remove any insoluble material.