Desmin

Item no.	20-512-300033
Manufacturer	GENWAY
Amount	0.1 mg
Category	Oncology Antibodies Cell Biology Cytoskeleton Primary Antibodies Intermediate filaments By Organ Testicular & Prostate Cancer
Type	Antibody
Clone	RD301
Specific against	other
ECLASS 10.1	32160702
ECLASS 11.0	32160702
UNSPSC	12352203
Alias	GWB-C3C37F
Similar products	20-512-300033
Available
Genway ID:	GWB-C3C37F
Subclass:	IgG2b
Clone:	RD301
Specificity:	RD301 reacts exclusively with desmin. which is expressed in smooth and striated muscle cells and their tumors e. g. rhabdomyosarcoma and leiomyosarcoma. RD301 is suitable for immunoblotting and immunohistochemistry on frozen tissues. Optimal antibody dilution should be determined by titration; recommended range is 1:100 ? 1:200 for immunohistochemistry with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent. and 1:100 ? 1:1000 for immunoblotting applications. Product
Note:	Each vial contains 100 ?l 1 mg/ml purified antibody in PBS containing 0. 09% sodium azide. Desmin (53 kDa) exhibits a high degree of tissue specificity. its expression being predominantly confined to all types of muscle cells (cardiac. skeletal and smooth muscle). Regulation of desmin expression is stage and tissue-specific. since it is induced during terminal development of. for example. skeletal muscle cell differentiation. In skeletal en cardiac muscle cells desmin is localized in the Z-disk region and at the intercalated disk. The expression pattern of desmin in smooth muscle is much more heterogenous. Coexpression of vimentin and desmin has been observed in tumors derived from muscle tissue. i. e. rhabdomyosarcomas and leiomyosarcomas. Furthermore. during myocard dysfunction dramatic changes in the distribution of desmin have been observed.
Function:	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.
Subunit:	Homopolymer.
Subcellular Location:	Cytoplasm.
Disease:	Defects in DES are the cause of desmin-related cardio-skeletal myopathy (CSM) [MIM:601419]; 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).
Disease:	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.
Disease:	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.
Similarity:	Belongs to the intermediate filament family. 1. Quax. W. van den Broek. L. Egberts. W. V. Ramaekers. F. and Bloemendal. H. (1985). Characterization of the hamster desmin gene: expression and formation of desmin filaments in nonmuscle cells after gene transfer. Cell 43. 327-38. 2. Verhagen. A. P. Aalders. T. W. Ramaekers. F. C. Debruyne. F. M. and Schalken. J. A. (1988). Differential expression of keratins in the basal and luminal compartments of rat prostatic epithelium during degeneration and regeneration. Prostate 13. 25-38. 3. Pieper. F. R. Schaart. G. Krimpenfort. P. J. Henderik. J. B. Moshage. H. J. van de Kemp. A. Ramaekers. F. C. Berns. A. and Bloemendal. H. (1989). Transgenic expression of the muscle-specific intermediate filament protein desmin in nonmuscle cells. J Cell Biol 108. 1009-24. 5. Raats. J. M. Pieper. F. R. Vree Egberts. W. T. Verrijp. K. N. Ramaekers. F. C. and Bloemendal. H. (1990). Assembly of aminoterminally deleted desmin in vimentin-free cells. J Cell Biol 111. 1971-85. 6. Schaart. G. Pieper. F. R. Kuijpers. H. J. Bloemendal. H. and Ramaekers. F. C. (1991). Baby hamster kidney (BHK-21/C13) cells can express striated muscle type proteins. Differentiation 46. 105-15. 7. Dispersyn. G. D. Geuens. E. Ver Donck. L. Ramaekers. F. C. and Borgers. M. (2001). Adult rabbit cardiomyocytes undergo hibernation-like dedifferentiation when cocultured with cardiac fibroblasts. Cardiovasc Res 51. 230-40.

Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.

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Amount: 0.1 mg

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