Product Description for Desmin
Desmin.
Presentation: Purified
Presentation: Purified
Properties for Desmin
| Product Category | Proteins & Growth Factors |
|---|---|
| Quantity | 0.1 mg |
| Synonyms | DES |
| Presentation | Purified |
| Molecular weight | 53 kDa |
| Source | Stomach |
| Species (Protein) | Chicken |
| Shipping to | Worldwide |
| PDF datasheet | View Datasheet |
| Manufacturer | OriGene Technologies GmbH |
| Material safety datasheet | MSDS for Proteins (de) |
Datasheet Extract
| Immunogen |
|
|---|---|
| Purity | > 98 % pure as determined by SDS-PAGE |
| Add. information | Isoelectric point: pHI 5.4 |
| Application | Protein standard in 1D and 2D SDS gel electrophoresis. Immunoassay. Antigen for immunization. |
| 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. |
| Concentration | 1.0 mg/ml. |
| General Readings |
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| Storage | Store lyophilized product at 2-8°C . After reconstitution store at -20°C. Avoid repeated freezing and thawing. Shelf life: one year from despatch. |
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