產品編號 yb-1176R
英文名稱 Anti-Agrin抗體
中文名稱 聚集蛋白抗體
別    名 AGRIN; Agrin proteoglycan; AGRN; FLJ45064; OTTHUMP00000044043; AGRIN_MOUSE.
Anti-Agrin抗體
說 明 書 0.1ml  0.2ml  
研究領域 細胞生物  神經生物學  信號轉導  細胞粘附分子  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat, 
產品應用 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 208kDa
細胞定位 細胞外基質 分泌型蛋白 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from mouse Agrin (1841-1940aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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
產品介紹 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 由運動神經分泌可誘導肌纖維的乙酰*酯酶和乙酰*受體聚集的蛋白；蛋白聚糖在發育中對于促進細胞間相互起著重要的作用。蛋白聚糖分布廣泛，并與神經系統的發育具有密切關系。