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      Rabbit Anti-Desmin  antibody (bs-33858R)
      ~~~促銷,代碼SSY221101~~~
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      產品編號 bs-33858R
      英文名稱 Desmin
      中文名稱 結蛋白抗體
      別    名 DESM_HUMAN; DES;   
      研究領域 腫瘤  心血管  免疫學  信號轉導  細胞類型標志物  
      抗體來源 Rabbit
      克隆類型 Polyclonal
      交叉反應 Human, Mouse, Rat, 
      產品應用 IHC-P=1:200-500 (石蠟切片需做抗原修復)
      not yet tested in other applications.
      optimal dilutions/concentrations should be determined by the end user.
      理論分子量 52kDa
      細胞定位 細胞漿 
      性    狀 Liquid
      濃    度 1mg/ml
      免 疫 原 Recombinant human Desmin protein 
      亞    型 IgG
      純化方法 affinity purified by Protein A
      緩 沖 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
      保存條件 Shipped at 4℃. Store at -20 °C 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 Desmin is a muscle-specific, type III intermediate filament that integrates the sarcolemma, Z disk, and nuclear membrane in sarcomeres and regulates sarcomere architecture. 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

      SwissProt: P17661 Human

      SwissProt: P31001 Mouse

      SwissProt: P48675 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 (human myocardium); 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) Polyclonal Antibody, Unconjugated (bs-33858R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
      Paraformaldehyde-fixed, paraffin embedded (rat skeletal muscle); 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) Polyclonal Antibody, Unconjugated (bs-33858R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
      Paraformaldehyde-fixed, paraffin embedded (mouse 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) Polyclonal Antibody, Unconjugated (bs-33858R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
      Paraformaldehyde-fixed, paraffin embedded (mouse skeletal muscle); 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) Polyclonal Antibody, Unconjugated (bs-33858R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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