Desmin (DES) Mouse Monoclonal Antibody [Clone ID: D33]

CAT#: AM32294SU-N

Desmin (DES) mouse monoclonal antibody, clone D33, Supernatant

Specifications

Product Data

• Clone Name: D33
• Applications: IF, IHC, WB
• Recommended Dilution: Western Blot: Use a preparation of leiomyoma cells as Positive Control.
  Immunofluorescence.
  Immunohistochemistry on Frozen Sections: ~1/100, preferably in PBS.
  Immunohistochemistry on Formalin Fixed Paraffin Embedded Sections: Microwave treatment is required.
  Recommended Positive Control: Skeleton muscle, rhabdomyosarcomas and leiomyoma cells.
• Reactivities: Human
• Host: Mouse
• Isotype: IgG1
• Clonality: Monoclonal
• Immunogen: Human leiomyoma.
• Specificity: This antibody is highly reactive with Desmin.
  On immunoblots only the 52 kD Desmin band is stained.
  The antibody stains muscle cells in Human tissues.
  Frozen sections show excellent staining in Immunohistochemistry or Immunofluorescence.
• Formulation: 0.15 M PBS
  State: Supernatant
  State: Liquid Tissue Culture Supernatant
  Stabilizer: 1% BSA
  Preservative: 0.09% Sodium Azide
• Concentration: lot specific
• Conjugation: Unconjugated
• Storage: Store undiluted at 2-8°C.
• Stability: Shelf life: one year from despatch
• Gene Name: desmin
• Database Link:
  Entrez Gene 1674 Human
  UniProt ID P17661
• Background: Desmins belongs to the intermediate filament family, and 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 Desmin 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 Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.
• Synonyms: DES