FITC标记的肌营养蛋白δ/δ-sarcoglycan抗体上海沪震实业有限公司

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产品名称: FITC标记的肌营养蛋白δ/δ-sarcoglycan抗体
英文名称: Anti-delta Sarcoglycan/FITC
抗体货号: HZ-14264R-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: ICC=1:50-200 IF=1:50-200
更新时间: 2018/10/11 10:54:00
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
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上海·中国-上海
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产品详细描述

 Rabbit Anti-delta Sarcoglycan/FITC Conjugated antibody

FITC标记的肌营养蛋白δ/δ-sarcoglycan抗体

 

英文名称 Anti-delta Sarcoglycan/FITC
中文名称 FITC标记的肌营养蛋白δ/δ-sarcoglycan抗体
别    名 35 kDa dystrophin associated glycoprotein; 35 kDa dystrophin-associated glycoprotein; 35DAG; CMD1L; DAGD; Delta-sarcoglycan; Delta-SG; Dystrophin associated glycoprotein delta sarcoglycan; LGMD2F; MGC22567; Placental delta sarcoglycan; Sarcoglycan delta (35 kDa dystrophin associated glycoprotein); SG delta; SGCD; SGCD_HUMAN; SGCDP; SGD.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  细胞生物  细胞骨架  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human delta Sarcoglycan
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:
The protein encoded by this gene is one of the four known components of the sarcoglycan complex, which is a subcomplex of the dystrophin-glycoprotein complex (DGC). DGC forms a link between the F-actin cytoskeleton and the extracellular matrix. This protein is expressed most abundantly in skeletal and cardiac muscle. Mutations in this gene have been associated with autosomal recessive limb-girdle muscular dystrophy and dilated cardiomyopathy. Alternatively spliced transcript variants encoding distinct isoforms have been observed for this gene. [provided by RefSeq, Jul 2008]

Function:
Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.

Subcellular Location:
Cell membrane > sarcolemma. Cytoplasm > cytoskelet

Tissue Specificity:
Most strongly expressed in skeletal and cardiac muscle. Also detected in smooth muscle. Weak expression in brain and lung.

Post-translational modifications:
Glycosylated.
Disulfide bonds are present.

DISEASE:
Defects in SGCD are the cause of limb-girdle muscular dystrophy type 2F (LGMD2F) [MIM:601287]. LGMD2F is an autosomal recessive disorder.
Defects in SGCD are the cause of cardiomyopathy dilated type 1L (CMD1L) [MIM:606685]. 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:
Belongs to the sarcoglycan beta/delta/gamma/zeta family.

Database links:

Entrez Gene: 6444 Human

Entrez Gene: 24052 Mouse

Omim: 601411 Human

SwissProt: Q92629 Human

SwissProt: P82347 Mouse

Unigene: 387207 Human

Unigene: 644733 Human

Unigene: 338890 Mouse



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

该基因编码的蛋白是肌糖复合物(dystrophin-glycoprotein.,DGC)的一个亚复合物,它是肌糖复合物的四种已知成分之一。DGC在F-肌动蛋白细胞骨架和细胞外基质之间形成联系。该蛋白在骨骼肌和心肌中表达最丰富。该基因突变与常染色体隐性遗传肢带肌营养不良和扩张型心肌病有关。选择性剪接转录变体编码DISTI

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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