结蛋白抗体

结蛋白抗体

规格：1mg/1ml

英文名： Desmin

别名： CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.

分子量： 52kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human Desmin

交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, Guinea Pig,

细胞定位：细胞浆

结蛋白抗体产品介绍：background: filaments found in muscle cells. In ***** 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 ***** 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结蛋白抗体 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结蛋白抗体 unspecific pathology to typical, myofibrillar changes with accumulation of desmin. Similarity: Belongs to the intermediate filament family. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 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**、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。

结蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 ICC=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.

研究领域：肿瘤  心血管  **学  信号转导  细胞类型标志物  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid

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