FITC标记的α肌萎缩糖蛋白2抗体
产品名称： FITC标记的α肌萎缩糖蛋白2抗体
英文名称： Anti-alpha Sarcoglycan/FITC
产品编号： HZ-8740R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
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Rabbit Anti-alpha Sarcoglycan/FITC Conjugated antibody
FITC标记的α肌萎缩糖蛋白2抗体
| 英文名称 | Anti-alpha Sarcoglycan/FITC |
| 中文名称 | FITC标记的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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 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 | Human, Mouse, Rat, Dog, Pig, Rabbit, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 40kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 2mg/1ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human alpha Sarcoglycan |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: 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. 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. |
肌糖复合物的组成部分，肌营养不良蛋白-糖蛋白复合物的亚复合物，在F-肌动蛋白细胞骨架和细胞外基质之间形成连接。