Product nameAnti-Desmin antibody [RD301]
See all Desmin primary antibodies
DescriptionMouse monoclonal [RD301] to Desmin
SpecificityThis antibody reacts exclusively with desmin, which is expressed in smooth and striated muscle cells and their tumors e.g. rhabdomyosarcoma and leiomyosarcoma.
Tested applicationsSuitable for: ICC/IF, IHC-Fr, WBmore details
Species reactivityReacts with: Mouse, Rat, Rabbit, Chicken, Hamster, Dog, Human, Pig
Does not react with: Zebrafish
Cytoskeletal desmin extract of chicken gizzard.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferPreservative: 0.09% Sodium azide
Concentration information loading...
PurityProtein A purified
Light chain typeunknown
Our Abpromise guarantee covers the use of ab8976 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use at an assay dependent concentration.
|IHC-Fr||1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent.|
|WB||1/100 - 1/1000.|
FunctionDesmin 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.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the intermediate filament family.
- Information by UniProt
- CMD1I antibody
- CSM1 antibody
- CSM2 antibody
This product has been referenced in:
- Ji HP et al. MicroRNA-28 potentially regulates the photoreceptor lineage commitment of Müller glia-derived progenitors. Sci Rep 7:11374 (2017). Read more (PubMed: 28900179) »
- Maiellaro-Rafferty K et al. Altered regional cardiac wall mechanics are associated with differential cardiomyocyte calcium handling due to nebulette mutations in preclinical inherited dilated cardiomyopathy. J Mol Cell Cardiol 60:151-60 (2013). WB ; Mouse . Read more (PubMed: 23632046) »