AGRN Antibody

中文名稱：

AGRN兔多克隆抗體
貨號：

CSB-PA001461LA01HU
規格：

￥440
圖片：
Immunofluorescence staining of Hela cells with CSB-PA001461LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

Western Blot
Positive WB detected in: A549 whole cell lysate, U87 whole cell lysate, 293 whole cell lysate
All lanes: AGRN antibody at 7.5μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 218, 206, 217, 215 kDa
Observed band size: 218 kDa

IHC image of CSB-PA001461LA01HU diluted at 1:400 and staining in paraffin-embedded human lung tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

IHC image of CSB-PA001461LA01HU diluted at 1:400 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
其他：

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靶點詳情

產品詳情

產品描述：

The product CSB-PA001461LA01HU is a rabbit polyclonal antibody raised against recombinant human AGRN (968-1130aa). This AGRN antibody is purified by protein G and gets a purity of up to 95%. It can recognize and detect human AGRN protein in ELISA, WB, IHC, and IF applications.

The AGRN protein mainly regulates the formation and maintenance of synapses between neurons and muscle cells. It has also been implicated in cell adhesion, migration, and proliferation. Dysregulation of AGRN expression or function can cause various pathological conditions, including muscular dystrophy, myasthenia gravis, and Alzheimer's disease.
產品名稱：

Rabbit anti-Homo sapiens (Human) AGRN Polyclonal antibody
Uniprot No.：

O00468
基因名：

AGRN
別名：

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
反應種屬：

Human
免疫原：

Recombinant Human Agrin protein (968-1130AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，AGRN Antibody (CSB-PA001461LA01HU)，的標記方式是Non-conjugated。對于AGRN Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA001461LB01HU	AGRN Antibody, HRP conjugated	ELISA
FITC	CSB-PA001461LC01HU	AGRN Antibody, FITC conjugated
Biotin	CSB-PA001461LD01HU	AGRN Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC, IF

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
IHC	1:200-1:500
IF	1:50-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
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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相關疾病：

Myasthenic syndrome, congenital, 8 (CMS8)
亞細胞定位：

[Isoform 1]: Secreted, extracellular space, extracellular matrix.; [Isoform 2]: Cell junction, synapse. Cell membrane; Single-pass type II membrane protein.
組織特異性：

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
數據庫鏈接：

HGNC: 329

OMIM: 103320

KEGG: hsa:375790

STRING: 9606.ENSP00000368678

UniGene: Hs.273330

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