Anti-desmin/FITC抗体说明书
Catalog Number : RSF-1026R
Quantity size : 0.1ml （2mg/ml; Reconstitute with 0.5 ml of sterile distilled water.）
Background:
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. Specificity :
· anti-desmin is a rabbit polyclonal antibody, FITC conjugated
· specific for desmin of human, mouse and rat
· epitope mapping near the amino terminus of desmin of human,
mouse rat origin
· human, mouse, rat cross reacted
· affinity chromatography purification, purity :>95%
· Isotype: IgG
· mol wt: 25kDa
Application :
· FCM: 1:20-100
· Immunohistochemistry : 1:20-200
·Immunofluorescence: 1:20-200
· Excitation spectrum: 490-495nm
· Emission spectrum: 520-530nm
· Optimal working dilutions must be determined by the end user.
Storage: Store at –20 ºC for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20ºC. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody, the antibody is stable for at least two weeks at 2-4 ºC.
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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