Anti-Dystrophin/FITC Conjugated抗体上海钰博生物科技有限公司

金牌供应商

性能参数

产品名称: Anti-Dystrophin/FITC Conjugated抗体
英文名称: Anti-Dystrophin/FITC
抗体货号: YB--6718R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human Dystrophin/DMD
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Dog, Pig, Horse, Rabbit,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/4/17 9:24:00
详细资料:  实验方法技术资料
浏览人数:7
诚信指数:1658点
了解更多:进入公司展台
使用范围:仅限科研使用,不能应用于临床
点此求购
在线QQ: 我是3002799160,请问我有什么可以帮到您呢?
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。

供应商联系卡

上海钰博生物科技有限公司
地址:
上海市沪闵路6088号龙之梦大厦8楼806室
邮编:
200612
电话:
021-60514606
传真:
021-60514606
联系人:
顾磊
所在区域:
上海·上海
邮件:
公司展台:
在线QQ:
我是3002799160,请问我有什么可以帮到您呢?
扫一扫,关注我们

产品详细描述

 Anti-Dystrophin/FITC Conjugated抗体

产品编号 YB-6718R-FITC
英文名称 Anti-Dystrophin/FITC
中文名称 FITC标记的肌营养不良蛋白抗体
别    名 Alternative namesApo dystrophin; BMD; CMD3B; DMD; DMD_HUMAN; Duchenne muscular dystrophy protein; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dystrophin; Muscular dystrophy Duchenne and Becker types.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  干细胞  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 405kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Dystrophin/DMD
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.
产品介绍 background:
Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.
Tissue specificityExpressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.

Function:
Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.

Subunit:
Interacts with SYNM (By similarity). Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Interacts with KRT19. Component of the dystrophin-associated glycoprotein complex which is composed of three subcomplexes: a cytoplasmic complex comprised of DMD (or UTRN), DTNA and a number of syntrophins, such as SNTB1, SNTB2, SNTG1 and SNTG2, the transmembrane dystroglycan complex, and the sarcoglycan-sarcospan complex. Interacts with DAG1 (betaDAG1) with DMD; the interaction is inhibited by phosphorylation on the PPXY motif of DAG1.

Subcellular Location:
Cell membrane; sarcolemma. Cytoplasm; cytoskeleton.

Tissue Specificity:
Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.

DISEASE:
Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment.
Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X-linked type 3B (CMD3B) [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). 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.

Similarity:
Contains 2 CH (calponin-homology) domains.
Contains 22 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger.

Database links:
 

UniProtKB/Swiss-Prot: P11532.3



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。

查看Anti-Dystrophin/FITC Conjugated抗体产品的用户还对以下产品感兴趣

暂无

ADVERTISEMENT

找不到您所需的产品,发布求购试试?

标题:*
描述:
姓名:*
Email:*
单位:*
电话:*
地址:*
邮编:
资料: 需要 不需要
报价: 需要 不需要