Product & ReviewsAntibodies

Desmin Antibody

Product Details

Cat. No.
251398
Type
Primary Antibody
Clonality
Polyclonal
Host
Rabbit
Abbiotec

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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 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 desmin 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.

Biological Information

  • Clonality: Polyclonal
  • Host: Rabbit
  • Reactivity: Human, Mouse, Rat
  • Antigen: KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human desmin.
  • Isotype: Rabbit Ig
  • Gene: P17661

Handling

  • Quantity: 0.1 mg
  • Storage: Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.

Applications

  • ELISA (ELISA)
  • Immunohistochemistry (IHC)
  • Western Blotting (WB)
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