DES is a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in its gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies.This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies.1) Fidzianska, A., (2005) Neuromuscul. Disord. 15 (8), 525-531.
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human DES.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAWB
DES antibody can be used for detection of DES by ELISA at 1:312500. DES antibody can be used for detection of DES by western blot at 5.0 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
DES antibody can be used for detection of DES by ELISA at 1:312500. DES antibody can be used for detection of DES by western blot at 5.0 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Additional Information
Form
Liquid
Storage Instructions
For short periods of storage (days) store at 4˚C. For longer periods of storage, store DES antibody at -20˚C. As with any antibody avoid repeat freeze-thaw cycles.
Storage Buffer
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.