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Rabbit Anti-alpha Sarcoglycan  antibody (bs-22471R)
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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-22471R
英文名称 alpha Sarcoglycan
中文名称 α肌萎缩糖蛋白2抗体
别    名 50 DAG; 50 kDa dystrophin associated glycoprotein; 50 kDa dystrophin-associated glycoprotein; 50DAG; 50kD DAG; 59kDa; A2; adhalin; ADL; Alpha SG; Alpha-sarcoglycan; Alpha-SG; Asg; DAG2; DMDA2; Dystroglycan 2; Dystroglycan-2; LGMD2D; sarcoglycan, alpha (dystrophin-associated glycoprotein); SCARMD1; Sgca; SGCA_HUMAN.  
研究领域 细胞生物  神经生物学  信号转导  糖蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,Rat (predicted: Human,Pig,Cow,Rabbit)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 40kDa
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human alpha Sarcoglycan: 211-310/387 <Extracellular>
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 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.

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).

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

Tissue Specificity:
Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain.

DISEASE:
Defects in SGCA are the cause of limb-girdle muscular dystrophy type 2D (LGMD2D) [MIM:608099]; also known as Duchenne-like muscular dystrophy autosomal recessive type 2 or severe childhood autosomal recessive muscular dystrophy (SCARMD). LGMD2D is an autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency. The phenotype is less severe than LGMD2C.

Similarity:
Belongs to the sarcoglycan alpha/epsilon family.

SWISS:
Q16586

Gene ID:
6442

Database links:

Entrez Gene: 6442 Human

Entrez Gene: 20391 Mouse

Entrez Gene: 303468 Rat

Omim: 600119 Human

SwissProt: Q16586 Human

SwissProt: P82350 Mouse

SwissProt: Q5SWB2 Mouse

SwissProt: Q28686 Rabbit

Unigene: 463412 Human

Unigene: 18709 Mouse

Unigene: 136653 Rat



产品图片
Sample:
Heart (Mouse) Lysate at 40 ug
Heart (Rat) Lysate at 40 ug
Kidney (Mouse) Lysate at 40 ug
Primary: Anti-alpha Sarcoglycan (bs-22471R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 50 kD
Observed band size: 50 kD
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