常规说明Desmin is the protein subunit of muscle-type intermediate filaments (IFs). IFs, with characteristic 10 nm diameter are a distinct class of heterogenous protein subunits apparent by both immunological and biochemical criteria. IFs differ significantly from the other cytoskeletal elements of the cell, namely microtubules & microfilaments, and are components of most eukaryotic cells. Desmin is one of the five major groups of IFs and is found in predominatly in skeletal, cardiac and smooth muscle.
Rabbit Anti-Desmin may be used for immunocyto-chemical localization of intermediate filaments of the desmin group in all types of muscle cells and to localize desmin at the periphery of z-discs. The product also specifically stains desmin when used in immunoblotting.
存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
纯化说明The antiserum has been treated to remove lipo-proteins.
Primary antibody说明Rabbit Anti-Desmin may be used for immunocyto-chemical localization of intermediate filaments of the desmin group in all types of muscle cells and to localize desmin at the periphery of z-discs. The product also specifically stains desmin when used in immunoblotting.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use at an assay dependent dilution.
功能Desmin 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.
疾病相关Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES) [MIM:601419]; also known as desmin-related myopathy (DRM). A 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 DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. 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. Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.