| 应用范围 | WB, |
| 抗体英文名 | alpha Sarcoglycan |
| 规格 | 100ul |
| 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. |
| 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 |
| 储 存 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. |
| 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 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. |
