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Desmin Rabbit pAb (bs-20702R)  
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產品編號 bs-20702R
英文名稱 Desmin Rabbit pAb
中文名稱 結蛋白抗體
別    名 DESM_HUMAN; DES;  
Specific References  (3)     |     bs-20702R 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.  
[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.  
[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.  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Rat (predicted: Human,Mouse,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 52 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Desmin: 311-400/470 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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在很多哺乳動物中的橫紋肌和各種平滑肌及其來源的腫瘤組織中都有表達。結蛋白是一種中間絲蛋白,廣泛分布于骨骼肌細胞、平滑肌細胞、心肌細胞和肌上皮細胞及其腫瘤中,主要用于子宮、皮膚、胃腸道及其它橫紋肌肉瘤和肌上皮瘤的診斷和鑒別診斷。
產品圖片
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 (bs-20702R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (bs-0295G-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 (bs-20702R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (bs-0295G-cy3) for 90 minutes and DAPI for nuclei staining.
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