RAPSN polyclonal antibody

The RAPSN gene locus is located on chromosome 11p11.1-p11.2 and encodes a peripheral membrane protein. Rapsyn (receptor-associated protein of the synapse) is expressed in the postsynaptic membrane of skeletal muscle. Rap-syn is required for the clustering of nicotinic acetylcholine receptors (nAChR). Rapsyn self-associates through at least two of its seven tetra-tricopeptide repeats (TPRs). Rapsyn interacts with the large intracellular domain of the nAChR a subunit through the hydrophobic surface of the coiled-coil domain. Rapsyn modifies trafficking of AChR within the cell. Expression is essential for agrin-induced AChR clustering. Overexpression inhibits agrin-induced AChR clustering pathway. Absence of rapsyn causes deficit in the formation of postsynaptic specializations at neuromuscular synapse, which increases axonal branching and motonueron survival. Rapsyn plays a role in selective targeting of newly synthesized intracellular AChR to postsynaptic membrane.