磷酸化结蛋白抗体上海沪震实业有限公司

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性能参数

产品名称: 磷酸化结蛋白抗体
英文名称: phospho-Desmin (Ser60)
抗体货号: hz-14327R
产品规格: 100ul
级    别: , , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 1580元
抗原: IgG
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 见说明书
应用范围: WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500
更新时间: 2018/8/25 15:42:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
地址:
上海市杨浦区密云路1018号复旦科技园808室
邮编:
200612
电话:
021-60345367
传真:
021-31320307
联系人:
陈先生
所在区域:
上海·中国-上海
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产品详细描述

 Rabbit Anti-phospho-Desmin (Ser60)  antibody 

 

产品编号 hz-14327R
英文名称 phospho-Desmin (Ser60)
中文名称 ***酸化结蛋白抗体
别    名 p-Desmin (phospho S60); Desmin (phospho S60); CMD1I; CSM1; CSM2; DES; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865.  
   
产品类型 ***酸化抗体 
研究领域 肿瘤  心血管  细胞生物  Alzheimer's  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.
分 子 量 10kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated Synthesised phosphopeptide derived from human DES around the phosphorylation site of Ser60:RT(p-S)GG 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
PubMed PubMed
产品介绍 background:
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.

Subcellular Location:
Cytoplasm.

DISEASE:
Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES) [MIM:601419]; also known as desmin-related myopathy (DRM). 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.
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.

Gene ID:
1674

Database links:

Entrez Gene: 1674 Human

Entrez Gene: 13346 Mouse

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.

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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