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Product Name Agrin Chinese Name 聚集蛋白抗体 Alias AGRIN; Agrin proteoglycan; AGRN; FLJ45064; OTTHUMP00000044043; AGRIN_HUMAN; AGRIN_HUMAN. Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Cow, Sheep, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 213kDa Cellular localization Extracellular matrix Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Agrin: 1151-1245/2045 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
SWISS:
O00468
Gene ID:
375790
Database links:Entrez Gene: 375790 Human
Entrez Gene: 11603 Mouse
SwissProt: O00468 Human
Omim: 103320 Human
SwissProt: A2ASQ1 Mouse
Unigene: 273330 Human
Unigene: 2163 Rat
Product Picture Tissue/cell: mouse colon tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-Agrin Polyclonal Antibody, Unconjugated(SL10219R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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