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Rabbit Anti-gamma Sarcoglycan  antibody (bs-16231R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-16231R
英文名称 gamma Sarcoglycan
中文名称 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.  
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 Rat (predicted: Human,Mouse,Dog,Pig,Cow,Sheep)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 32kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human gamma Sarcoglycan: 151-250/291 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
Q13326

Gene ID:
6445

Database links:

Entrez Gene: 6445 Human

Omim: 608896 Human

SwissProt: Q13326 Human

Unigene: 37167 Human



产品图片
Tissue/cell: Rat myocardium tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-SGCG Polyclonal Antibody, Unconjugated(bs-16231R) 1:500, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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