Product Name	Desmin
Chinese Name	结蛋白抗体
Alias	DESM_HUMAN; DES;
literatures	Specific References  (3)     |     SL20702R has been referenced in 3 publications. [IF=5.959] Wang Y et al. SPARCL1 promotes C2C12 cell differentiation via BMP7-mediated BMP/TGF-β cell signaling pathway. Cell Death Dis. 2019 Nov 7;10(11):852.  WB ;  Mouse.   PubMed:31699966 [IF=3.759] Yanjun Duan. et al. MicroRNA-214 Inhibits Chicken Myoblasts Proliferation, Promotes Their Differentiation, and Targets the TRMT61A Gene. Genes-Basel. 2020 Dec;11(12):1400  IF ;  Chicken.   PubMed:33255823 [IF=3.231] Pengfei Shi. et al. Analysis of Promoter Methylation of the Bovine FOXO1 Gene and Its Effect on Proliferation and Differentiation of Myoblasts. ANIMALS. 2023 Jan;13(2):319  IF ;  Bovine.   PubMed:36670858
Research Area	Tumour  Cell biology  immunology  Signal transduction  Cell type markers
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	52kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Desmin: 311-400/470
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) 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). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin. 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. Interacts with DST. Interacts with MTM1. Subcellular Location: Cytoplasm. Post-translational modifications: ADP-ribosylation prevents ability to form intermediate filaments. DISEASE: Defects in DES are the cause of myopathy myofibrillar type 1 (MFM1) [MIM:601419]. 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. Note=Mutations in the DES gene are associated with a variable clinical phenotype which encompasses isolated myopathies, pure cardiac phenotypes (including dilated cardiomyopathy, restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy), cardiac conduction disease, and combinations of these disorders. If both cardiologic and neurologic features occur, they can manifest in any order, as cardiologic features can precede, occur simultaneously with, or follow manifestation of generalized neuromuscular disease (PubMed:19879535). 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. Similarity: Belongs to the intermediate filament family. SWISS: P17661 Gene ID: 1674 Database links: Entrez Gene: 1674 Human Entrez Gene: 13346 Mouse Entrez Gene: 64362 Rat Omim: 125660 Human SwissProt: P17661 Human SwissProt: P31001 Mouse SwissProt: P48675 Rat Unigene: 594952 Human Unigene: 6712 Mouse Unigene: 39196 Rat Desmin在很多哺乳动物中的横纹肌和各种平滑肌及其来源的Tumour组织中都有表达。结蛋白是一种中间丝蛋白，广泛分布于骨骼肌细胞、平滑肌细胞、心肌细胞和肌epithelial cells及其Tumour中，主要用于子宫、皮肤、胃肠道及其它横纹肌肉瘤和肌上皮瘤的诊断和鉴别诊断。
Product Picture	Paraformaldehyde-fixed, paraffin embedded (Rat heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Desmin; DES) Polyclonal Antibody, Unconjugated (SL20702R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (SL0295G-cy3) for 90 minutes. Paraformaldehyde-fixed, paraffin embedded (Rat heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Desmin; DES) Polyclonal Antibody, Unconjugated (SL20702R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (SL0295G-cy3) for 90 minutes and DAPI for nuclei staining. Blank control (black line): A431(black) (The cells were fixed with 2% paraformaldehyde (10 min) , then permeabilized with PBST for 30 min on room temperature) Primary Antibody (green line): Rabbit Anti-Desmin antibody (SL3152R) ; Dilution: 1μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody (white blue line): Goat anti-rabbit IgG-FITC;Dilution: 1μg /test.

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