聚集蛋白抗体

聚集蛋白抗体英文名称  Anti-Agrin 

中文名称  聚集蛋白抗体 

别    名  AGRIN; Agrin proteoglycan; AGRN; FLJ45064; OTTHUMP00000044043; AGRIN_HUMAN; AGRIN_MOUSE. 

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg  

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Mouse, Rat  

产品类型  一抗    

研究领域  细胞生物 神经生物学 信号转导 细胞粘附分子 细胞类型标志物  

蛋白分子量  predicted molecular weight: 208kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from mouse Agrin (1841-1940aa) 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   ELISA=1:500-1000  IP=1:20-100  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.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

聚集蛋白抗体产品介绍 Agrin is a neuronal aggregating factor that induces the aggregation of acetylcholine receptors and other postsynaptic proteins on muscle fibers and is crucial for the formation of the neuromuscular junction. Agrin is an essential extracellular matrix component which promotes clustering of nicotinic acetylcholine receptors (nAChRs) and other proteins during development at the neuromuscular junction. Agrin, MuSK and Rapsyn are all essential components for AChR aggregation, through an unknown mechanism. The C-terminal region of agrin is released into the medium, interacts with receptors on the muscle surface and induces AChR aggregation. The central region contains two O-linked glycosylation sites and a domain homologous to domain III of laminin. The N-terminal region anchors agrin to the extracellular matrix via other basal membrane components. This region also contains a protease inhibitor domain and glycosaminoglycan attachment sites; increasing the predicted MW from 200kDa to ~600kDa.

Function : Plays a central role in the formation and the maintenance of the neuromuscular junction (NMJ), the synapse between motor neuron and skeletal muscle. Ligand of the MUSK signaling complex that directly binds LRP4 in this complex and induces the phosphorylation of MUSK, the kinase of the complex. The activation of MUSK in myotubes induces the formation of NMJ by regulating different processes including the transcription of specific genes and the clustering of AChR in the postsynaptic membrane.

Subunit : Interacts with LRP4; the interaction is direct and recruits AGRIN to the MUSK signaling complex composed at least of MUSK and LRP4 (By similarity). Binds to laminin.

Subcellular Location : Secreted, extracellular space, extracellular matrix. Note=Synaptic basal lamina at the neuromuscular junction.

Tissue Specificity : Detected on the aortic endothelium (at protein level).

Post-translational modifications : Contains heparan sulfate chains as well as N-linked and O-linked oligosaccharides. 0-fucosylation of EGF repeat 4 of muscle AGRN by POFUT1 prevents its ability to form AChR clusters.

DISEASE : Defects in AGRN are a cause of myasthenia, limb-girdle, familial (LGM) [MIM:254300]. A congenital myasthenic syndrome characterized by a typical 'limb girdle' pattern of muscle weakness with small, simplified neuromuscular junctions but normal acetylcholine receptor and acetylcholinesterase function.

Similarity : Contains 4 EGF-like domains.

Contains 9 Kazal-like domains. 

Contains 2 laminin EGF-like domains. 

Contains 3 laminin G-like domains. 

Contains 1 NtA (N-terminal agrin) domain. 

Contains 1 SEA domain.

Database links : UniProtKB/Swiss-Prot: O00468.4 humna

UniProtKB/Swiss-Prot: A2ASQ1.1 mouse

神经元标志物（Neuron marker)