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

Specifications

Product Data
Clone Name	D33
Applications	IF, IHC, WB
Recommend 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.
Reactivity	Human
Host	Mouse
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 Condition	Store undiluted at 2-8°C.
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
Reference Data

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