Desmin (10H7) Antibody
Product Details
- Cat. No.
- 252674
- Type
- Primary Antibody
- Clonality
- Monoclonal
- Host
- Mouse
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Description
Desmin is a class-III intermediate filament found in muscle cells. In adult striated muscle desmin forms 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 myopathy myofibrillar desmin-related (MFM-DES), also known as desmin-related myopathy (DRM). DRM is 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.
Biological Information
- Clonality: Monoclonal
- Host: Mouse
- Reactivity: Human
- Antigen: Purified recombinant fragment of Desmin expressed in E. coli.
- Clone: 10H7D2
- Isotype: Mouse IgG1
- Gene: P17661
Handling
- Quantity: 0.1 mg
- Storage: Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
Applications
- Immunohistochemistry (IHC)
- Western Blotting (WB)