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AGRN Antibody

  • 货号：
    CSB-PA801486
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA801486(AGRN Antibody) at dilution 1/50, on the right is treated with synthetic peptide. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    O00468
  • 基因名：
    AGRN
  • 别名：
    AGRIN antibody; Agrin C-terminal 22 kDa fragment antibody; Agrin proteoglycan antibody; AGRIN_HUMAN antibody; Agrn antibody; C22 antibody; C90 antibody; FLJ45064 antibody; OTTHUMP00000044043 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthetic peptide of Human AGRN
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:3000-1:10000
    IHC 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    heparan sulfate basal lamina glycoprotein that plays a central role in the formation and the maintenance of the neuromuscular junction (NMJ) and directs key events in postsynaptic differentiation. Component of the AGRN-LRP4 receptor complex that induces the phosphorylation and activation of MUSK. 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. Calcium ions are required for maximal AChR clustering. AGRN function in neurons is highly regulated by alternative splicing, glycan binding and proteolytic processing. Modulates calcium ion homeostasis in neurons, specifically by inducing an increase in cytoplasmic calcium ions. Functions differentially in the central nervous system (CNS) by inhibiting the alpha(3)-subtype of Na+/K+-ATPase and evoking depolarization at CNS synapses. This secreted isoform forms a bridge, after release from motor neurons, to basal lamina through binding laminin via the NtA domain.; transmembrane form that is the predominate form in neurons of the brain, induces dendritic filopodia and synapse formation in mature hippocampal neurons in large part due to the attached glycosaminoglycan chains and the action of Rho-family GTPases.; Isoform 1, isoform 4 and isoform 5: neuron-specific (z+) isoforms that contain C-terminal insertions of 8-19 AA are potent activators of AChR clustering. Isoform 5, agrin (z+8), containing the 8-AA insert, forms a receptor complex in myotubules containing the neuronal AGRN, the muscle-specific kinase MUSK and LRP4, a member of the LDL receptor family. The splicing factors, NOVA1 and NOVA2, regulate AGRN splicing and production of the 'z' isoforms.; Isoform 3 and isoform 6: lack any 'z' insert, are muscle-specific and may be involved in endothelial cell differentiation.; is involved in regulation of neurite outgrowth probably due to the presence of the glycosaminoglcan (GAG) side chains of heparan and chondroitin sulfate attached to the Ser/Thr- and Gly/Ser-rich regions. Also involved in modulation of growth factor signaling.; this released fragment is important for agrin signaling and to exert a maximal dendritic filopodia-inducing effect. All 'z' splice variants (z+) of this fragment also show an increase in the number of filopodia.
  • 基因功能参考文献：
    1. High Agrin expression is associated with Chronic obstructive pulmonary disease. PMID: 29351440
    2. The presence of agrin at locations with particular importance for the growth and stability of atherosclerotic plaques renders this molecule strategically positioned to influence plaque development and vulnerability PMID: 29405249
    3. C-terminal agrin fragment levels predict acute kidney injury after acute myocardial infarction. PMID: 28646861
    4. Agrin promotes oncogenesis through YAP-dependent transcription. PMID: 28273460
    5. Agrin strongly promotes chondrocyte differentiation and cartilage formation in vivo. PMID: 26290588
    6. Knockdown of agrin and perlecan promoted a decrease on cell migration and adhesion, and on resistance of cells to cisplatin. PMID: 25506919
    7. Among 42 hip fractured patients (age 83.7+/-8.6 years, 76.2% women), sarcopenia was diagnosed in 7 individuals (16.7%). Serum C-terminal agrin fragment (CAF) levels were significantly higher in sarcopenic relative to non-sarcopenic patients. PMID: 25304331
    8. In patients suffering from severe sepsis and septic shock, serum levels of C-terminal agrin fragment were significantly associated with kidney function and the need for renal replacement therapy and were not influenced by severe septic conditions. PMID: 25807640
    9. MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin-independent AChR clusters PMID: 24244707
    10. these observations indicate that agrin is another autoantigen in patients with MG and agrin autoantibodies may be pathogenic through inhibition of agrin/LRP4/MuSK signaling at the NMJ. PMID: 24632822
    11. Five new recessive mutations in the gene encoding agrin are identified in patients with congenital myasthenic syndrome. PMID: 24951643
    12. study identifies a spontaneous agrin mutation that reduces the ability of z+ agrin to activate MuSK and induce AChR clustering; this results in a severe congenital myasthenic syndrome in the patient, with both pre- and postsynaptic defects at the neuromuscular junction PMID: 22205389
    13. Dynamics of expression patterns of agrin in human glioblastoma PMID: 22307776
    14. In contrast to wild-type neurons which form synapses and survive for prolonged periods, agrin-deficient neurons do not mature and are rapidly eliminated in the transgenic olfactory bulb. PMID: 22423096
    15. Agrin immunohistochemistry may facilitate determination of primary versus metastatic origin in problematic liver cancer cases. PMID: 20471664
    16. different regions within the agrin protein are responsible for synapse formation at the neuromuscular junction and for process formation in central nervous system neurons PMID: 19940118
    17. Agrin, a heparan sulfate proteoglycan, is a component of the basal lamina of BBB microvessels, and growing evidence suggests that it may be important for the maintenance of the BBB. PMID: 12070669
    18. acts at the nerve-muscle synapse in the glomerular basal membrane and on T-lymphocytes PMID: 12073527
    19. evidence for additional functions of agrin during axonal growth, establishment of the blood-brain barrier, and Alzheimer's disease is accumulating--REVIEW PMID: 12270958
    20. Thus, an agrin/MuSK complex may form part of a motor neuron stop signal involved in "reverse signaling" to the motor neuron. PMID: 15691710
    21. in the NtA-laminin complex, conserved amino acids in the gamma 1 chain are prerequisite for the binding to agrin PMID: 15694127
    22. agrin has a role in binding alpha-synuclein and modulating alpha-synuclein fibrillation PMID: 16037493
    23. In human sperm an agrin isoform with a short NH2-terminus (agrinSN) localized in the posterior post-acrosomal, neck, and flagellar mid-piece regions. PMID: 16487930
    24. agrin might play an important role in neoangiogenesis in human HCC, being a part of the newly formed vasculature. In CC, however, agrin might be involved in tumor progression PMID: 17640714
    25. The agrin expression in human T cells is regulated by cell activation and IFN-alpha, and may have an important function during cell activation with potential implications for autoimmunity. PMID: 18025246
    26. study concludes that Abeta can modulate the cellular expression of agrin and glypican-1, which may contribute to the accumulation of these heparan sulfate proteoglycans in Alzheimer's disease lesions PMID: 19166823
    27. agrin facilitates the discrimination of benign and malignant hepatocellular lesions PMID: 19194276
    28. The agrin mutation does not interfere with its ability to induce postsynaptic structures but that it dramatically perturbs the maintenance of the neuromuscular junction. PMID: 19631309
    29. Observational study of gene-disease association. (HuGE Navigator) PMID: 19598235

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  • 相关疾病：
    Myasthenic syndrome, congenital, 8 (CMS8)
  • 亚细胞定位：
    [Isoform 1]: Secreted, extracellular space, extracellular matrix.; [Isoform 2]: Cell junction, synapse. Cell membrane; Single-pass type II membrane protein.
  • 组织特异性：
    Expressed in basement membranes of lung and kidney. Muscle- and neuron-specific isoforms are found. Isoforms (y+) with the 4 AA insert and (z+8) isoforms with the 8 AA insert are all neuron-specific. Isoforms (z+11) are found in both neuronal and non-neur
  • 数据库链接：

    HGNC: 329

    OMIM: 103320

    KEGG: hsa:375790

    STRING: 9606.ENSP00000368678

    UniGene: Hs.273330