| 產品編號 | bsm-60266R |
| 英文名稱 | Rabbit Anti-Desmin antibody |
| 中文名稱 | 結蛋白重組兔單抗 |
| 別 名 | CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; FLJ12025; FLJ39719; FLJ41013; FLJ41793. |
| 研究領域 | 腫瘤 心血管 免疫學 信號轉導 |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 交叉反應 | Rat,Mouse,Human |
| 產品應用 | WB=1:500-2000, IHC-P=1:200-1:1000, ICC=1:50, 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 | 52kDa |
| 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 |
| 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 | This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008] 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 SwissProt: P17661 Human SwissProt: P31001 Mouse |
| 產品圖片 |  Sample: Lane 1: Mouse Heart tissue lysates Lane 2: Mouse Muscle tissue lysates Primary: Anti-Desmin (bsm-60266R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 52 kDa Observed band size: 54 kDa  Paraformaldehyde-fixed, paraffin embedded (human endometrial carcinoma); 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; Incubation with (Desmin) Monoclonal Antibody, Unconjugated (bsm-60266R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (Human 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; Incubation with (Desmin) Monoclonal Antibody, Unconjugated (bsm-60266R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (Human colon); 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; Incubation with (Desmin) Monoclonal Antibody, Unconjugated (bsm-60266R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (Human 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; Incubation with (Desmin) Monoclonal Antibody, Unconjugated (bsm-60266R) 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) Monoclonal Antibody, Unconjugated (bsm-60266R) 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 stomach); 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) Monoclonal Antibody, Unconjugated (bsm-60266R) 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) Monoclonal Antibody, Unconjugated (bsm-60266R) 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 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) Monoclonal Antibody, Unconjugated (bsm-60266R) 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 stomach); 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) Monoclonal Antibody, Unconjugated (bsm-60266R) 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) Monoclonal Antibody, Unconjugated (bsm-60266R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.  Cell line: C2C12 Fixative: 4% Paraformaldehyde Permeabilization: 0.1% TritonX-100 Primary ab dilution: 1:50 Primary incubation condition: 4°C overnight Secondary ab: Goat Anti-Rabbit IgG Nuclear counter stain: DAPI (Blue) Comment: Color green is the positive signal for bsm-60266R |
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