Rapsn

Summary

Gene Symbol: Rapsn
Description: receptor-associated protein of the synapse
Alias: 43kDa, Nraps, Raps, rapsyn, 43 kDa receptor-associated protein of the synapse, 43 kDa postsynaptic protein, 43kDa acetylcholine receptor-associated protein, acetylcholine receptor-associated 43 kDa protein, receptor-associated protein of the synapse, 43 kDa
Species: mouse
Products:     Rapsn

Top Publications

  1. Gervasio O, Armson P, Phillips W. Developmental increase in the amount of rapsyn per acetylcholine receptor promotes postsynaptic receptor packing and stability. Dev Biol. 2007;305:262-75 pubmed
    ..was a concurrent two-fold increase in immunofluorescent labeling for the AChR-associated cytoplasmic protein, rapsyn. When 1-month old muscle was denervated, postsynaptic rapsyn immunostaining was reduced, as was the efficiency of ..
  2. Feng G, Steinbach J, Sanes J. Rapsyn clusters neuronal acetylcholine receptors but is inessential for formation of an interneuronal cholinergic synapse. J Neurosci. 1998;18:4166-76 pubmed
    ..A cytoplasmic protein, rapsyn, is essential for AChR clustering in muscle...
  3. Chen F, Qian L, Yang Z, Huang Y, Ngo S, Ruan N, et al. Rapsyn interaction with calpain stabilizes AChR clusters at the neuromuscular junction. Neuron. 2007;55:247-60 pubmed
    ..Interestingly, the AChR-associated protein rapsyn interacted with calpain in an agrin-dependent manner, and this interaction inhibited the protease activity of ..
  4. Zhang B, Luo S, Dong X, Zhang X, Liu C, Luo Z, et al. Beta-catenin regulates acetylcholine receptor clustering in muscle cells through interaction with rapsyn. J Neurosci. 2007;27:3968-73 pubmed
    ..Here we demonstrate that the beta-catenin interacts with rapsyn, a molecule key for AChR clustering...
  5. Noakes P, Phillips W, Hanley T, Sanes J, Merlie J. 43K protein and acetylcholine receptors colocalize during the initial stages of neuromuscular synapse formation in vivo. Dev Biol. 1993;155:275-80 pubmed
    ..The precise colocalization of 43K and AChRs persisted through development. These results are consistent with 43K being involved in the nerve-induced clustering of AChRs during synapse formation. ..
  6. Herbst R, Avetisova E, Burden S. Restoration of synapse formation in Musk mutant mice expressing a Musk/Trk chimeric receptor. Development. 2002;129:5449-60 pubmed
    ..Moreover, acetylcholine receptor clustering and motor terminal branching are restored in parallel, indicating that the extent of presynaptic differentiation is matched to the extent of postsynaptic differentiation. ..
  7. Weatherbee S, Anderson K, Niswander L. LDL-receptor-related protein 4 is crucial for formation of the neuromuscular junction. Development. 2006;133:4993-5000 pubmed
    ..The identification of Lrp4 as a crucial factor for NMJ formation may have implications for human neuromuscular diseases such as myasthenia syndromes. ..
  8. Gautam M, Noakes P, Mudd J, Nichol M, Chu G, Sanes J, et al. Failure of postsynaptic specialization to develop at neuromuscular junctions of rapsyn-deficient mice. Nature. 1995;377:232-6 pubmed
    ..A key component of this complex is rapsyn, a peripheral membrane protein of relative molecular mass 43K (refs 4, 5), which is precisely colocalized with ..
  9. Luo S, Zhang B, Dong X, Tao Y, Ting A, Zhou Z, et al. HSP90 beta regulates rapsyn turnover and subsequent AChR cluster formation and maintenance. Neuron. 2008;60:97-110 pubmed publisher
    b>Rapsyn, an acetylcholine receptor (AChR)-interacting protein, is essential for synapse formation at the neuromuscular junction (NMJ). Like many synaptic proteins, rapsyn turns over rapidly at synapses...

More Information

Publications54

  1. Gautam M, Noakes P, Moscoso L, Rupp F, Scheller R, Merlie J, et al. Defective neuromuscular synaptogenesis in agrin-deficient mutant mice. Cell. 1996;85:525-35 pubmed
  2. Misgeld T, Burgess R, Lewis R, Cunningham J, Lichtman J, Sanes J. Roles of neurotransmitter in synapse formation: development of neuromuscular junctions lacking choline acetyltransferase. Neuron. 2002;36:635-48 pubmed
    ..At subsequent stages, neurotransmission delays some steps in synaptic maturation but accelerates others. Thus, neurotransmission affects synaptogenesis from early stages and coordinates rather than drives synaptic maturation. ..
  3. Wang J, Fu X, Lei W, Wang T, Sheng A, Luo Z. Nuclear factor kappaB controls acetylcholine receptor clustering at the neuromuscular junction. J Neurosci. 2010;30:11104-13 pubmed publisher
    ..and requires a number of intracellular signal or structural proteins, including AChR-associated scaffold protein Rapsyn. Here, we report a role of nuclear factor kappaB (NF-kappaB), a well known transcription factor involved in a ..
  4. Arikawa Hirasawa E, Rossi S, Rotundo R, Yamada Y. Absence of acetylcholinesterase at the neuromuscular junctions of perlecan-null mice. Nat Neurosci. 2002;5:119-23 pubmed
  5. Banks G, Chau T, Bartlett S, Noakes P. Promotion of motoneuron survival and branching in rapsyn-deficient mice. J Comp Neurol. 2001;429:156-65 pubmed
    ..of synapse formation on motoneuron survival during embryonic development, we counted the number of motoneurons in rapsyn-deficient mice...
  6. Osman A, Schrader A, Hawkes A, Akil O, Bergeron A, Lustig L, et al. Muscle-like nicotinic receptor accessory molecules in sensory hair cells of the inner ear. Mol Cell Neurosci. 2008;38:153-69 pubmed publisher
    ..MuSK, rapsyn and RIC-3 are accessory molecules associated with muscle and brain nAChR function...
  7. Smith C, Mittaud P, Prescott E, Fuhrer C, Burden S. Src, Fyn, and Yes are not required for neuromuscular synapse formation but are necessary for stabilization of agrin-induced clusters of acetylcholine receptors. J Neurosci. 2001;21:3151-60 pubmed
    ..These data show that the stabilization of agrin-induced AChR clusters requires Src and Fyn and suggest that the adaptor activities, rather than the kinase activities, of these kinases are essential for this stabilization. ..
  8. Gautam M, Mudd J, Copeland N, Gilbert D, Jenkins N, Merlie J. Characterization and mapping of the Rapsn gene encoding the 43-kDa acetylcholine receptor-associated protein. Genomics. 1994;24:366-9 pubmed
    ..Finally, the 43K locus, designated Rapsn, has been mapped to the central region of mouse chromosome 2.
  9. Nguyen Q, Son Y, Sanes J, Lichtman J. Nerve terminals form but fail to mature when postsynaptic differentiation is blocked: in vivo analysis using mammalian nerve-muscle chimeras. J Neurosci. 2000;20:6077-86 pubmed
    ..nerves entered transplanted muscles derived from mice lacking muscle-specific receptor tyrosine kinase (MuSK) or rapsyn, muscle-specific components required for postsynaptic differentiation...
  10. Sigoillot S, Bourgeois F, Lambergeon M, Strochlic L, Legay C. ColQ controls postsynaptic differentiation at the neuromuscular junction. J Neurosci. 2010;30:13-23 pubmed publisher
    ..MuSK promotes acetylcholine receptor (AChR) clustering in a process mediated by rapsyn, a cytoplasmic protein that stimulates AChR packing in clusters and regulates synaptic gene transcription...
  11. Li L, Cao Y, Wu H, Ye X, Zhu Z, Xing G, et al. Enzymatic Activity of the Scaffold Protein Rapsyn for Synapse Formation. Neuron. 2016;92:1007-1019 pubmed publisher
    ..This is mediated by scaffold proteins that bridge the receptors with cytoskeleton. One such protein is rapsyn (receptor-associated protein at synapse), which is essential for acetylcholine receptor (AChR) clustering and NMJ (..
  12. Pun S, Sigrist M, Santos A, Ruegg M, Sanes J, Jessell T, et al. An intrinsic distinction in neuromuscular junction assembly and maintenance in different skeletal muscles. Neuron. 2002;34:357-70 pubmed
    ..Our results show that postsynaptic differentiation processes intrinsic to FaSyn and DeSyn muscles influence the formation of NMJs during development and their maintenance in the adult. ..
  13. Rodova M, Kelly K, VanSaun M, Daniel J, Werle M. Regulation of the rapsyn promoter by kaiso and delta-catenin. Mol Cell Biol. 2004;24:7188-96 pubmed
    b>Rapsyn is a synapse-specific protein that is required for clustering acetylcholine receptors at the neuromuscular junction...
  14. Liu Y, Padgett D, Takahashi M, Li H, Sayeed A, Teichert R, et al. Essential roles of the acetylcholine receptor gamma-subunit in neuromuscular synaptic patterning. Development. 2008;135:1957-67 pubmed publisher
    ..branching, increased motoneuron survival, as well as aberrant distribution of acetylcholinesterase (AChE) and rapsyn. However, clustering of muscle specific kinase (MuSK) proceeded normally in the gamma-null muscles...
  15. Dobbins G, Luo S, Yang Z, Xiong W, Mei L. alpha-Actinin interacts with rapsyn in agrin-stimulated AChR clustering. Mol Brain. 2008;1:18 pubmed publisher
    ..We show that ?-actinin, a protein known to cross-link F-actin, interacts with rapsyn, a scaffold protein essential for neuromuscular junction formation...
  16. Ettorre M, Lorenzetto E, Laperchia C, Baiguera C, Branca C, Benarese M, et al. Glutamatergic neurons induce expression of functional glutamatergic synapses in primary myotubes. PLoS ONE. 2012;7:e31451 pubmed publisher
    ..showed that typical anchoring proteins of central excitatory synapses coimmunoprecipitate and colocalize with rapsyn, the acetylcholine receptor anchoring protein at the neuromuscular junction...
  17. Mihailovska E, Raith M, Valencia R, Fischer I, Al Banchaabouchi M, Herbst R, et al. Neuromuscular synapse integrity requires linkage of acetylcholine receptors to postsynaptic intermediate filament networks via rapsyn-plectin 1f complexes. Mol Biol Cell. 2014;25:4130-49 pubmed publisher
    ..We found plectin isoform 1f (P1f) to bridge AChRs and IFs via direct interaction with the AChR-scaffolding protein rapsyn in an isoform-specific manner; forced expression of P1f in plectin-deficient cells rescued both compromised AChR ..
  18. Borges L, Yechikhov S, Lee Y, Rudell J, Friese M, Burden S, et al. Identification of a motif in the acetylcholine receptor beta subunit whose phosphorylation regulates rapsyn association and postsynaptic receptor localization. J Neurosci. 2008;28:11468-76 pubmed publisher
    ..the acetylcholine receptor (AChR) is specifically clustered in the postsynaptic membrane via interactions with rapsyn and other scaffolding proteins...
  19. Froehner S. Expression of RNA transcripts for the postsynaptic 43 kDa protein in innervated and denervated rat skeletal muscle. FEBS Lett. 1989;249:229-33 pubmed
    ..Thus, the expression of these two proteins is regulated by different mechanisms. ..
  20. Lei K, Zhang X, Ding X, Guo X, Chen M, Zhu B, et al. SUN1 and SUN2 play critical but partially redundant roles in anchoring nuclei in skeletal muscle cells in mice. Proc Natl Acad Sci U S A. 2009;106:10207-12 pubmed publisher
    ..These results clearly indicate that SUN1 and SUN2 function critically in skeletal muscle cells for Syne-1 localization at the NE, which is essential for proper myonuclear positioning. ..
  21. Lee Y, Rudell J, Yechikhov S, Taylor R, Swope S, Ferns M. Rapsyn carboxyl terminal domains mediate muscle specific kinase-induced phosphorylation of the muscle acetylcholine receptor. Neuroscience. 2008;153:997-1007 pubmed publisher
    ..by agrin signaling via the muscle specific kinase (MuSK) and requires an intracellular scaffolding protein called rapsyn. In addition to its structural role, rapsyn is also necessary for agrin-induced tyrosine phosphorylation of the ..
  22. Gervasio O, Phillips W. Increased ratio of rapsyn to ACh receptor stabilizes postsynaptic receptors at the mouse neuromuscular synapse. J Physiol. 2005;562:673-85 pubmed
    ..The AChR-associated protein rapsyn is essential for forming AChR clusters during development...
  23. An M, Lin W, Yang J, Dominguez B, Padgett D, Sugiura Y, et al. Acetylcholine negatively regulates development of the neuromuscular junction through distinct cellular mechanisms. Proc Natl Acad Sci U S A. 2010;107:10702-7 pubmed publisher
    ..Together, these data suggest that ACh negatively regulates axon growth and presynaptic specialization at the neuromuscular junction through distinct cellular mechanisms. ..
  24. Chen P, Martinez Pena y Valenzuela I, Aittaleb M, Akaaboune M. AChRs Are Essential for the Targeting of Rapsyn to the Postsynaptic Membrane of NMJs in Living Mice. J Neurosci. 2016;36:5680-5 pubmed publisher
    b>Rapsyn, a 43 kDa scaffold protein, is required for the clustering of acetylcholine receptors (AChRs) at synaptic sites between mammalian motor neurons and muscle cells...
  25. Wang J, Ruan N, Qian L, Lei W, Chen F, Luo Z. Wnt/beta-catenin signaling suppresses Rapsyn expression and inhibits acetylcholine receptor clustering at the neuromuscular junction. J Biol Chem. 2008;283:21668-75 pubmed publisher
    ..we report that Wnt3a negatively regulates acetylcholine receptor (AChR) clustering by repressing the expression of Rapsyn, an AChR-associated protein essential for AChR clustering...
  26. Antolik C, Catino D, Resneck W, Bloch R. The tetratricopeptide repeat domains of rapsyn bind directly to cytoplasmic sequences of the muscle-specific kinase. Neuroscience. 2006;141:87-100 pubmed
    ..Acetylcholine receptor clustering is also dependent on the postsynaptic scaffolding protein, rapsyn, which binds to acetylcholine receptors...
  27. Wang Z, Washabaugh C, Yao Y, Wang J, Zhang L, Ontell M, et al. Aberrant development of motor axons and neuromuscular synapses in MyoD-null mice. J Neurosci. 2003;23:5161-9 pubmed
    ..However, MyoD-/- mice assemble a complex postsynaptic apparatus that includes muscle-specific kinase (MuSK), rapsyn, erbB, and utrophin.
  28. Marchand S, Devillers Thiery A, Pons S, Changeux J, Cartaud J. Rapsyn escorts the nicotinic acetylcholine receptor along the exocytic pathway via association with lipid rafts. J Neurosci. 2002;22:8891-901 pubmed
    The 43 kDa receptor-associated protein rapsyn is a myristoylated peripheral protein that plays a central role in nicotinic acetylcholine receptor (AChR) clustering at the neuromuscular junction...
  29. Lin W, Sanchez H, Deerinck T, Morris J, Ellisman M, Lee K. Aberrant development of motor axons and neuromuscular synapses in erbB2-deficient mice. Proc Natl Acad Sci U S A. 2000;97:1299-304 pubmed
    ..The postsynaptic defect features an impairment of junctional folds at the neuromuscular synapse in the mutants. These results demonstrate that erbB2 is essential for in vivo development of the NMJ. ..
  30. Frail D, McLaughlin L, Mudd J, Merlie J. Identification of the mouse muscle 43,000-dalton acetylcholine receptor-associated protein (RAPsyn) by cDNA cloning. J Biol Chem. 1988;263:15602-7 pubmed
    ..The deduced sequence of mouse RAPsyn has 412 amino acids and a molecular mass of 46,392 daltons...
  31. Lee Y, Rudell J, Ferns M. Rapsyn interacts with the muscle acetylcholine receptor via alpha-helical domains in the alpha, beta, and epsilon subunit intracellular loops. Neuroscience. 2009;163:222-32 pubmed publisher
    ..is regulated by the motoneuron-derived factor, agrin, and requires an intracellular, scaffolding protein called rapsyn. However, it remains unclear where rapsyn binds on the acetylcholine receptor and how their interaction is ..
  32. Silver A, Moody J, Dunford R, Clark D, Ganz S, Bulman R, et al. Molecular mapping of chromosome 2 deletions in murine radiation-induced AML localizes a putative tumor suppressor gene to a 1.0 cM region homologous to human chromosome segment 11p11-12. Genes Chromosomes Cancer. 1999;24:95-104 pubmed
    ..YAC contig construction, together with data suggesting that the critical gene flanks Sfpi1, represents significant progress toward identifying an AML tumor suppressor gene. ..
  33. Musil L, Frail D, Merlie J. The mammalian 43-kD acetylcholine receptor-associated protein (RAPsyn) is expressed in some nonmuscle cells. J Cell Biol. 1989;108:1833-40 pubmed
    ..electric organ and vertebrate neuromuscular junctions contain the receptor-associated protein of the synapse (RAPsyn) (previously referred to as the 43K protein), a nonactin, 43,000-Mr peripheral membrane protein associated with ..
  34. Schwander M, Shirasaki R, Pfaff S, Muller U. Beta1 integrins in muscle, but not in motor neurons, are required for skeletal muscle innervation. J Neurosci. 2004;24:8181-91 pubmed
    ..We conclude that Itgb1 expression in muscle, but not in motor neurons, is critical for NMJ development. ..
  35. Banks G, Choy P, Lavidis N, Noakes P. Neuromuscular synapses mediate motor axon branching and motoneuron survival during the embryonic period of programmed cell death. Dev Biol. 2003;257:71-84 pubmed
    ..We achieved this by comparing agrin and rapsyn mutant mice that lack acetylcholine receptor (AChR) clusters...
  36. Terrado J, Burgess R, DeChiara T, Yancopoulos G, Sanes J, Kato A. Motoneuron survival is enhanced in the absence of neuromuscular junction formation in embryos. J Neurosci. 2001;21:3144-50 pubmed
    ..e., null mutants for agrin, nerve-derived agrin, rapsyn, and MuSK)...
  37. Herrmann D, Straubinger M, Hashemolhosseini S. Protein kinase CK2 interacts at the neuromuscular synapse with Rapsyn, Rac1, 14-3-3γ, and Dok-7 proteins and phosphorylates the latter two. J Biol Chem. 2015;290:22370-84 pubmed publisher
    ..the nicotinic acetylcholine receptors with weak interaction, (b) dishevelled (Dsh), and (c) another four proteins, Rapsyn, Rac1, 14-3-3γ, and Dok-7, with strong interaction...
  38. Frail D, Musil L, Buonanno A, Merlie J. Expression of RAPsyn (43K protein) and nicotinic acetylcholine receptor genes is not coordinately regulated in mouse muscle. Neuron. 1989;2:1077-86 pubmed
    b>RAPsyn (also known as 43K protein), a mouse muscle protein localized to the synaptic membrane, is thought to be involved in the localization of nicotinic acetylcholine receptors at the neuromuscular junction...
  39. Michalk A, Stricker S, Becker J, Rupps R, Pantzar T, Miertus J, et al. Acetylcholine receptor pathway mutations explain various fetal akinesia deformation sequence disorders. Am J Hum Genet. 2008;82:464-76 pubmed publisher
    ..subunits alpha1, beta1, and delta (CHRNA1, CHRNB1, CHRND) as well as receptor-associated protein of the synapse (RAPSN) previously revealed missense or compound nonsense-missense mutations in viable congenital myasthenic syndrome; ..
  40. Piguet J, Schreiter C, Segura J, Vogel H, Hovius R. Acetylcholine receptor organization in membrane domains in muscle cells: evidence for rapsyn-independent and rapsyn-dependent mechanisms. J Biol Chem. 2011;286:363-9 pubmed publisher
    ..b>Rapsyn plays a central role in directing and clustering nAChR during cellular differentiation and neuromuscular junction ..
  41. Gautam M, DeChiara T, Glass D, Yancopoulos G, Sanes J. Distinct phenotypes of mutant mice lacking agrin, MuSK, or rapsyn. Brain Res Dev Brain Res. 1999;114:171-8 pubmed
    ..junction requires agrin, a nerve-derived signal; MuSK, a critical component of the agrin receptor in muscle; and rapsyn, a protein that interacts with acetylcholine receptors (AChRs)...
  42. Brockhausen J, Cole R, Gervasio O, Ngo S, Noakes P, Phillips W. Neural agrin increases postsynaptic ACh receptor packing by elevating rapsyn protein at the mouse neuromuscular synapse. Dev Neurobiol. 2008;68:1153-69 pubmed publisher
    ..aggregates was reduced in 4-week postnatal mice that had reduced amounts of the AChR-associated protein, rapsyn, in the postsynaptic membrane (rapsyn(+/-) mice)...
  43. Choi K, Berrera M, Reischl M, Strack S, Albrizio M, Röder I, et al. Rapsyn mediates subsynaptic anchoring of PKA type I and stabilisation of acetylcholine receptor in vivo. J Cell Sci. 2012;125:714-23 pubmed publisher
    ..Here, we show that the AChR-associated protein, rapsyn, interacts with PKA type I in C2C12 and T-REx293 cells as well as in live mouse muscle beneath the neuromuscular ..
  44. Apel E, Glass D, Moscoso L, Yancopoulos G, Sanes J. Rapsyn is required for MuSK signaling and recruits synaptic components to a MuSK-containing scaffold. Neuron. 1997;18:623-35 pubmed
    ..The receptor tyrosine kinase MuSK is a component of the agrin receptor, while the cytoplasmic protein rapsyn is necessary for the clustering of AChRs and all other postsynaptic membrane components studied to date...
  45. Bruneau E, Akaaboune M. The dynamics of the rapsyn scaffolding protein at individual acetylcholine receptor clusters. J Biol Chem. 2007;282:9932-40 pubmed
    b>Rapsyn, a cytoplasmic receptor-associated protein, is required for the clustering of acetylcholine receptors (AChRs). Although AChR dynamics have been extensively studied, little is known about the dynamics of rapsyn...