Ab12364 binds to an epitope close to splicing site Z and causes a marked reduction in the number of nicotinic acetylcholine receptor(nAChR) clusters in samples from mouse and rat origins.
Mouse Brain Tissue Extract.
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Agrin is an essential extracellular matrix component which promotes clustering of nicotinic acetylcholine receptors (nAChRs) and other proteins during development at the neuromuscular junction. Agrin, MuSK and Rapsyn are all essential components for AChR aggregation, through an unknown mechanism. The C-terminal region of agrin is released into the medium, interacts with receptors on the muscle surface and induces AChR aggregation. The central region contains two O-linked glycosylation sites and a domain homologous to domain III of laminin. The N-terminal region anchors agrin to the extracellular matrix via other basal membrane components. This region also contains a protease inhibitor domain and glycosaminoglycan attachment sites; increasing the predicted MW from 200kDa to ~600kDa.
Secreted; extracellular space; extracellular matrix. Note: Synaptic basal lamina at the neuromuscular junction.