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α肌萎縮糖蛋白2抗體
  • 產品貨號：
    BN40694R
  • 中文名稱：
    α肌萎縮糖蛋白2抗體
  • 英文名稱：
    Rabbit anti-alpha Sarcoglycan Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產品規(guī)格

    售價

    備注

  • BN40694R-100ul

    100ul

    ￥2360.00

    交叉反應：Mouse,Rat(predicted:Human,Pig,Cow,Rabbit) 推薦應用：WB

  • BN40694R-200ul

    200ul

    ￥3490.00

    交叉反應：Mouse,Rat(predicted:Human,Pig,Cow,Rabbit) 推薦應用：WB

產品描述

英文名稱alpha Sarcoglycan
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Mouse, Rat,  (predicted: Human, Pig, Cow, Rabbit, )
產品應用WB=1:500-2000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
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:211-310/387 <Extracellular>
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產品介紹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



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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