| 產(chǎn)品編號 | bs-22471R |
| 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(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 糖蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse,Rat (predicted: Human,Rabbit,Pig,Cow) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 40kDa |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)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 |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | 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 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 (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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