Anti-Desmin Antibody - C-terminal (RMAB-0250520)
Cat. No.: RMAB-0250520
Category: Primary Antibodies
INQUIRY
100 μL
10 µL
Rabbit monoclonal to Desmin - C-terminal
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | SP138 |
| Form | Liquid |
| Purity | Protein A/G purified |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Synthetic peptide. |
| Applications | ICC/IF, WB, IHC-P |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Desmin - C-terminal; Suitable for: ICC/IF, WB, IHC-P; Reacts with: Mouse, Rat, Human |
Target Information
| Target Symbol | DES |
|---|---|
| Target Name | Desmin |
| UniProt ID | P17661 |
| Cellular Localization | Cytoplasm. |
| 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. |
| Involvement in Disease | Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES); also known as desmin-related myopathy (DRM). A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome). Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin. |
| Sequence Similarities | Belongs to the intermediate filament family. |
Storage & Shipping
| Storage Buffer | pH: 7.60; Preservative: 0.1% Sodium azide; Constituents: PBS, 1% BSA |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
