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Rabbit Anti-alpha Sarcoglycan  antibody (bs-8740R)  
~~~促銷代碼KT202411~~~
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產(chǎn)品編號 bs-8740R
英文名稱 Rabbit Anti-alpha Sarcoglycan  antibody
中文名稱 α肌萎縮糖蛋白2抗體
別    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.  
研究領域 細胞生物  神經(jīng)生物學  信號轉導  糖蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat (predicted: Human,Mouse,Rabbit,Pig,Dog)
產(chǎn)品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 40kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human alpha Sarcoglycan: 51-150/387 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



產(chǎn)品圖片
Sample: Heart(Rat) Lysate at 40 ug Primary: Anti- alpha Sarcoglycan (bs-8740R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 40 kD Observed band size: 40 kD
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