Anti-Agrin抗体聚集蛋白 agrin 由运动神经分泌可诱导肌纤维的乙酰*酯酶和乙酰*受体聚集的蛋白；蛋白聚糖在发育中对于促进细胞间相互起着重要的作用。蛋白聚糖分布广泛，并与神经系统的发育具有密切关系。

产品编号 yb-1176R
英文名称 Anti-Agrin抗体
中文名称 聚集蛋白抗体
别    名 AGRIN; Agrin proteoglycan; AGRN; FLJ45064; OTTHUMP00000044043; AGRIN_MOUSE.
Anti-Agrin抗体
说 明 书 0.1ml  0.2ml  
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  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse, Rat, 
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.
分 子 量 208kDa
细胞定位 细胞外基质 分泌型蛋白 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
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 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.

PubMed PubMed
产品介绍 background:
This gene encodes one of several proteins that are critical in the development of the neuromuscular junction (NMJ), as identified in mouse knock-out studies. The encoded protein contains several laminin G, Kazal type serine protease inhibitor, and epidermal growth factor domains. Additional post-translational modifications occur to add glycosaminoglycans and disulfide bonds. In one family with congenital myasthenic syndrome affecting limb-girdle muscles, a mutation in this gene was found. [provided by RefSeq, Aug 2011].

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.

聚集蛋白 agrin 由运动神经分泌可诱导肌纤维的乙酰*酯酶和乙酰*受体聚集的蛋白；蛋白聚糖在发育中对于促进细胞间相互起着重要的作用。蛋白聚糖分布广泛，并与神经系统的发育具有密切关系。