Key features and details
- Mouse monoclonal [RD301] to Desmin
- Suitable for: IHC-Fr
- Reacts with: Mouse, Rat, Rabbit, Chicken, Hamster, Dog, Human, Pig
- Isotype: IgG2b
製品名Anti-Desmin antibody [RD301]
Desmin 一次抗体 製品一覧
製品の詳細Mouse monoclonal [RD301] to Desmin
特異性This antibody reacts exclusively with desmin, which is expressed in smooth and striated muscle cells and their tumors e.g. rhabdomyosarcoma and leiomyosarcoma.
アプリケーション適用あり: IHC-Frmore details
種交差性交差種: Mouse, Rat, Rabbit, Chicken, Hamster, Dog, Human, Pig
Cytoskeletal desmin extract of chicken gizzard.
保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
バッファーPreservative: 0.09% Sodium azide
Concentration information loading...
精製度Protein A purified
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.
|IHC-Fr||1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent.|
機能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.
関連疾患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.
配列類似性Belongs to the intermediate filament family.
- Information by UniProt
- CMD1I antibody
- CSM1 antibody
- CSM2 antibody
ab8976 は 8 報の論文で使用されています。
- Wang S et al. H3K27me3 Depletion during Differentiation Promotes Myogenic Transcription in Porcine Satellite Cells. Genes (Basel) 10:N/A (2019). PubMed: 30893875
- Huang J et al. The Effect of µ/m-Calpain on Protein Degradation of Chicken Breast Meat. J Food Sci 84:1054-1059 (2019). PubMed: 31042817
- Niu F et al. Cocaine-induced release of CXCL10 from pericytes regulates monocyte transmigration into the CNS. J Cell Biol 218:700-721 (2019). PubMed: 30626719
- Ji HP et al. MicroRNA-28 potentially regulates the photoreceptor lineage commitment of Müller glia-derived progenitors. Sci Rep 7:11374 (2017). PubMed: 28900179
- Rodansky ES et al. Intestinal organoids: a model of intestinal fibrosis for evaluating anti-fibrotic drugs. Exp Mol Pathol 98:346-51 (2015). PubMed: 25828392
- Fu W et al. Comparative proteomic analysis of the breast muscle response to chronic corticosterone administration in broiler chickens showing long or short tonic immobility. Poult Sci 93:784-93 (2014). PubMed: 24706954
- Lal H et al. Caveolin and ß1-integrin coordinate angiotensinogen expression in cardiac myocytes. Int J Cardiol 168:436-45 (2013). PubMed: 23058350
- 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 . PubMed: 23632046