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当前位置: 上海基免 > 生物分子 > 结蛋白抗体

结蛋白抗体

供货周期: 7天
品牌: Abcam
型号: 0.2ml/200μg
货号:
报价: ¥1
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产品介绍
结蛋白抗体英文名称  Anti-Desmin/DES 
中文名称  结蛋白抗体 
别    名  CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793. 
公司结蛋白抗体应用于医学免疫学、动物免疫学、分子生物学、生物化学、临床医学、检验医学、动物医学、药学、理工、农学、环境学等生命科学密切相关的专业。  
浓    度  1mg/1ml 
规 格  0.1ml/100μg  0.2ml/200μg          
抗体来源  Rabbit  
克隆类型  polyclonal 
交叉反应  Human, Mouse, Rat, Chicken, Dog, Pig, Horse, Rabbit  
产品类型  一抗    
研究领域  肿瘤 心血管 免疫学 信号转导  
蛋白分子量  predicted molecular weight: 52kDa 
性    状  Lyophilized or Liquid 
免 疫 原  KLH conjugated synthetic peptide derived from human Desmin 
亚    型  IgG 
纯化方法  affinity purified by Protein A 
储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 
产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 
(石蜡切片需做抗原修复) 
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user.  
保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 
结蛋白抗体产品介绍 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.
Database links : UniProtKB/Swiss-Prot: P17661.3
Desmin在很多哺乳动物中的横纹肌和各种平滑肌及其来源的肿瘤组织中都有表达。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、心肌细胞和肌上皮细胞及其肿瘤中,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。
【存储要求】连续使用时4°C存储,保质期六个月;结蛋白抗体期存储时建议分装为10ul以上小包装-20°C存储,并避免反复冻融,保质期一年。
实验的用途:
1)WB:Western Blotting 免疫印迹
2)IH:Immunohistochemistry 免疫组化
3)IH(P):Immunohistochemistry Parraffin sections 免疫组化(石蜡)
4)IH(F):Immunohistochemistry Frozen sections 免疫组化(冰冻)
5)IC:Immunocytochemistry 免疫细胞化学
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上海基免实业有限公司

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310116002801941

成立日期

2013-03-11

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结蛋白抗体由上海基免实业有限公司为您提供,货号,规格:0.2ml/200μg ,CAS号:,如您想了解更多关于结蛋白抗体价格、结蛋白抗体结构式、批发、用途等信息,欢迎咨询。除供应结蛋白抗体外,还可为您提供犬细小病毒(CPV)检测试剂盒、蘑菇丝氨酸蛋白酶(PRSS)检测试剂盒、植物N-甲基腐胺氧化酶(MPO)检测试剂盒等试剂,公司有专业的客户服务团队,是您值得信赖的合作伙伴,上海基免客户服务电话,售前、售后均可联系。
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