Code
OSD00020W
$240USD
ID Tag
Rb895-260409-WS
Immunogen
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.
Accession
Also known
Des
Target
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.
Storage
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.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
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)
Limitation
For research use only
Related
products
Code
OSD00020W
$240USD
Unit size
150 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
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.
Specificity
Specific for Desmin.
Spcs X-react.
Mouse, human, rat. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 150 µl of sterile water. Centrifuge to remove any insoluble material.