Product nameAnti-Desmin antibody [D33]
See all Desmin primary antibodies
DescriptionMouse monoclonal [D33] to Desmin
SpecificityThis antibody is highly reactive with desmin. On immunoblots only the 56 kD desmin band is stained. Non reactive with GFAP, Keratin, Vimentin and Neurofilament.
Tested applicationsSuitable for: IHC-FoFr, IHC-Fr, IHC-P, WB, ICC/IFmore details
Species reactivityReacts with: Rat, Chicken, Hamster, Human
Tissue, cells or virus. Immunogen derived from human Leiomyoma.
Database link: P17661
General notesSufficient antibody for 100-500 tests
This antibody stains muscle cells in human tissues
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.1% Sodium azide
Constituent: 1% BSA
PurityTissue culture supernatant
Primary antibody notesThis antibody stains muscle cells in human tissues
Our Abpromise guarantee covers the use of ab8470 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent concentration. Predicted molecular weight: 53 kDa.|
|ICC/IF||Use at an assay dependent concentration. PubMed: 21699414|
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
IHC image of ab8470 staining in human skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab8470, 1µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
This product has been referenced in:
- Zhang XY et al. Regeneration of diaphragm with bio-3D cellular patch. Biomaterials 167:1-14 (2018). IHC ; Rat . Read more (PubMed: 29550580) »
- Spinazzola JM & Gussoni E Isolation of Primary Human Skeletal Muscle Cells. Bio Protoc 7:N/A (2017). Read more (PubMed: 29152538) »