α肌萎缩糖蛋白2抗体
规格：1mg/1ml
英文名： alpha Sarcoglycan
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; Dystrog
分子量： 40kDa
储存液：Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human alpha Sa
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆 细胞膜
α肌萎缩糖蛋白2抗体产品介绍：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) α肌萎缩糖蛋白2抗体[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.α肌萎缩糖蛋白2抗体 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.
α肌萎缩糖蛋白2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 信号转导 糖蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid