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Rabbit Anti-gamma Sarcoglycan/BF647 Conjugated antibody (bs-16231R-BF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-16231R-BF647
英文名称1 Rabbit Anti-gamma Sarcoglycan/BF647 Conjugated antibody
中文名称 BF647标记的γ-肌聚糖/肌聚糖​​伽玛/γ-sarcoglycan抗体
别    名 35 kDa dystrophin associated glycoprotein; 35 kDa dystrophin-associated glycoprotein; 35DAG; 35kD dystrophin associated glycoprotein; 35kDa dystrophin-associated glycoprotein; A4; DAGA4; DMDA; DMDA1; Gamma SG; Gamma-sarcoglycan; Gamma-SG; LGMD2C; MAM; MGC130048; Sarcoglycan gamma; SCARMD2; SCG3; SGCG; SGCG_HUMAN; TYPE.  
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  信号转导  细胞骨架  跨膜蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, 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 gamma 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:
This gene encodes gamma-sarcoglycan, one of several sarcolemmal transmembrane glycoproteins that interact with dystrophin. The dystrophin-glycoprotein complex (DGC) spans the sarcolemma and is comprised of dystrophin, syntrophin, alpha- and beta-dystroglycans and sarcoglycans. The DGC provides a structural link between the subsarcolemmal cytoskeleton and the extracellular matrix of muscle cells. Defects in the encoded protein can lead to early onset autosomal recessive muscular dystrophy, in particular limb-girdle muscular dystrophy, type 2C (LGMD2C). [provided by RefSeq, Oct 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.

Subunit:
Interacts with the syntrophin SNTA1. Cross-link to form 2 major subcomplexes: one consisting of SGCB, SGCD and SGCG and the other consisting of SGCB and SGCD. The association between SGCB and SGCG is particularly strong while SGCA is loosely associated with the other sarcoglycans (By similarity). Interacts with FLNC.

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

Tissue Specificity:
Expressed in skeletal and heart muscle.

DISEASE:
Defects in SGCG are the cause of limb-girdle muscular dystrophy type 2C (LGMD2C) [MIM:253700]. LGMD2C is characterized by progressive muscle wasting from early childhood.

Similarity:
Belongs to the sarcoglycan beta/delta/gamma/zeta family.

Database links:

Entrez Gene: 6445 Human

Omim: 608896 Human

SwissProt: Q13326 Human

Unigene: 37167 Human



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