伴肌动蛋白抗体
规格：1mg/1ml
英文名： Nebulin
别名： DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505.
分子量： 500-800kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
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 Nebulin
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit,
细胞定位：细胞浆
伴肌动蛋白抗体产品介绍：background: Nebulin is a high molecular weight protein (500-800 kD), localized at the N2-line of skeletal muscle myofibrils. It exhibits an elastic stretch dependence, as it maintains the same proportional distance between the z-line and the m-line as the sarcomere is stretched. Function: This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Bind and stabilize F-actin. Subcellular Location: Cytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton. Tissue Specificity: Muscle specific. Located in the thin filament of striated muscle. DISEASE: Defects in NEB are the cause of伴肌动蛋白抗体 nemaline myopathy type 2 (NEM2) [MIM:256030]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. Similarity: Contains 178 nebulin repeats. Contains 1 SH3 domain. Gene ID: 4703 Database links: Entrez Gene: 374027 Chicken Entrez Gene: 伴肌动蛋白抗体4703 Human Entrez Gene: 17996 Mouse Entrez Gene: 311029 Rat Omim: 161650 Human SwissProt: P20929 Human Unigene: 588655 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 具有稳定细肌丝的作用。
伴肌动蛋白抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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 **学 结合蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid