FITC标记的神经肌肉接头蛋白SNTA1抗体上海沪震实业有限公司

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产品名称: FITC标记的神经肌肉接头蛋白SNTA1抗体
英文名称: Anti-Syntrophin Alpha1/FITC
抗体货号: HZ-3600R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: IF=1:50-200
更新时间: 2018/8/29 13:38:00
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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产品详细描述

 Rabbit Anti-Syntrophin Alpha1/FITC Conjugated antibody 

FITC标记的神经肌肉接头蛋白SNTA1抗体

 

英文名称 Anti-Syntrophin Alpha1/FITC
中文名称 FITC标记的神经肌肉接头蛋白SNTA1抗体
别    名 SNT1; SNT2; SNT3; SNTA1; SNTB1; SNTB2; Syntrophin alpha 1; Syntrophin beta 1; Syntrophin beta 2.  
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  免疫学  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Pig, Cow, 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 56kDa
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 Syntrophins/SNTA1
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:
Syntrophins are cytoplasmic peripheral membrane scaffold proteins that are components of the dystrophin-associated protein complex. This gene is a member of the syntrophin gene family and encodes the most common syntrophin isoform found in cardiac tissues. The N-terminal PDZ domain of this syntrophin protein interacts with the C-terminus of the pore-forming alpha subunit (SCN5A) of the cardiac sodium channel Nav1.5. This protein also associates cardiac sodium channels with the nitric oxide synthase-PMCA4b (plasma membrane Ca-ATPase subtype 4b) complex in cardiomyocytes. This gene is a susceptibility locus for Long-QT syndrome (LQT) - an inherited disorder associated with sudden cardiac death from arrhythmia - and sudden infant death syndrome (SIDS). This protein also associates with dystrophin and dystrophin-related proteins at the neuromuscular junction and alters intracellular calcium ion levels in muscle tissue. [provided by RefSeq, Jan 2013].

Function:
Adapter protein that binds to and probably organizes the subcellular localization of a variety of membrane proteins. May link various receptors to the actin cytoskeleton and the extracellular matrix via the dystrophin glycoprotein complex. Plays an important role in synapse formation and in the organization of UTRN and acetylcholine receptors at the neuromuscular synapse. Binds to phosphatidylinositol 4,5-bisphosphate.

Subunit:
Monomer and homodimer. Interacts with the other members of the syntrophin family SNTB1 and SNTB2; SGCG and SGCA of the dystrophin glycoprotein complex; NOS1; GRB2; the sodium channel proteins SCN4A and SCN5A; F-actin and calmodulin. Interacts with dystrophin protein DMD and related proteins DTNA and UTRN and with MAPK12, TGFA and GA.

Subcellular Location:
Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cell junction. Cytoplasm, cytoskeleton. Note=In skeletal muscle, it localizes at the cytoplasmic side of the sarcolemmal membrane and at neuromuscular junctions.

Tissue Specificity:
High expression in skeletal muscle and heart. Low expression in brain, pancreas, liver, kidney and lung. Not detected in placenta.

Post-translational modifications:
Phosphorylated by CaM-kinase II. Phosphorylation may inhibit the interaction with DMD.

DISEASE:
Defects in SNTA1 are the cause of long QT syndrome type 12 (LQT12) [MIM:612955]. A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to excercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy.

Similarity:
Belongs to the syntrophin family. 
Contains 1 PDZ (DHR) domain. 
Contains 2 PH domains. 
Contains 1 SU (syntrophin unique) domain.

Database links:

Entrez Gene: 6640 Human

Entrez Gene: 6641 Human

Entrez Gene: 6645 Human

SwissProt: Q13424 Human

SwissProt: Q13425 Human

SwissProt: Q13884 Human

SwissProt: Q61234 Mouse

Unigene: 31121 Human

Unigene: 1541 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   

合成营养素是胞质外周膜支架蛋白,是肌营养不良蛋白相关蛋白复合物的组成部分。该基因是合成酶基因家族的成员,编码心脏组织中发现的最常见的同型异构酶异构体。该合成蛋白的N-端PDZ结构域与心脏钠通道Nav1.5的孔形成α亚基(SCN5A)的C-末端相互作用。该蛋白还将心肌钠通道与心肌细胞中的一氧化氮合酶PMCA4B(质膜Ca- ATPase亚型4B)复合物相关联。该基因是长QT综合征(LQT)的易感基因位点,是一种遗传性心律失常与猝死婴儿猝死综合征(SIDS)相关的遗传性疾病。这种蛋白质也与肌营养不良蛋白和肌营养不良蛋白相关蛋白在神经肌肉接头处相关,并改变肌肉组织中的细胞内钙离子水平。[ RefSeq,2013年1月]提供。

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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