聚集蛋白抗体
规格：1mg/1ml
英文名： Agrin
别名： AGRIN; Agrin proteoglycan; AGRN; FLJ45064; OTTHUMP; AGRIN_HUMAN; AGRIN_HUMAN.
分子量： 213kDa
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 Agrin
交叉反应：Human, Cow, Sheep,
细胞定位：细胞外基质 分泌型蛋白
聚集蛋白抗体产品介绍：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 聚集蛋白抗体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 聚集蛋白抗体(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: Entrez Gene: 375790 Human Entrez Gene: 11603 Mouse Entrez Gene: 25592 Rat SwissProt: O00468 Human Omim: 103320 Human SwissProt: A2ASQ1 Mouse SwissProt: P25304 Rat Unigene: 273330 Human Unigene: 2163 Rat 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：
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid