motor endplate

Summary

Summary: The specialized postsynaptic region of a muscle cell. The motor endplate is immediately across the synaptic cleft from the presynaptic axon terminal. Among its anatomical specializations are junctional folds which harbor a high density of cholinergic receptors.

Top Publications

  1. Urbano F, Piedras Rentería E, Jun K, Shin H, Uchitel O, Tsien R. Altered properties of quantal neurotransmitter release at endplates of mice lacking P/Q-type Ca2+ channels. Proc Natl Acad Sci U S A. 2003;100:3491-6 pubmed
    ..Changes in presynaptic function were also associated with a significant reduction in the size of postsynaptic acetylcholine receptor clusters. ..
  2. Tjust A, Brannstrom T, Pedrosa Domellöf F. Unaffected motor endplate occupancy in eye muscles of ALS G93A mouse model. Front Biosci (Schol Ed). 2012;4:1547-55 pubmed
    ..We propose that EOM-specific properties make these muscles more resistant to the underlying pathophysiological process of ALS and that the EOMs are a useful model to advance our understanding of ALS...
  3. Iino S, Kobayashi S, Hidaka H. Neurocalcin-immunopositive nerve terminals in the muscle spindle, Golgi tendon organ and motor endplate. Brain Res. 1998;808:294-9 pubmed
    ..These findings suggest that neurocalcin is distributed and regulates calcium signaling in both afferent and efferent nerve terminals in the muscles and tendons. ..
  4. Punga A, Nygren I, Askmark H, Stalberg E. Monozygous twins with neuromuscular transmission defects at opposite sides of the motor endplate. Acta Neurol Scand. 2009;119:207-11 pubmed publisher
    ..To our knowledge this is the first case report of its kind and adds an intriguing contrast to the more common diagnosis of CMS in monozygous twins...
  5. Van der Kloot W, Molgo J, Cameron R, Colasante C. Vesicle size and transmitter release at the frog neuromuscular junction when quantal acetylcholine content is increased or decreased. J Physiol. 2002;541:385-93 pubmed
    ..In the controls it was 97 micros. Perhaps some of the added ACh is bound within the vesicles, which slows the rise. Our major conclusion is that ACh content can change notably without any change in the size of the synaptic vesicles. ..
  6. Masuda A, Shen X, Ito M, Matsuura T, Engel A, Ohno K. hnRNP H enhances skipping of a nonfunctional exon P3A in CHRNA1 and a mutation disrupting its binding causes congenital myasthenic syndrome. Hum Mol Genet. 2008;17:4022-35 pubmed publisher
    ..Our findings imply that the presence of the hnRNP H-binding motif close to the 3' end of an intron is an essential but underestimated splicing regulator of the downstream exon. ..
  7. Cifuentes Diaz C, Nicole S, Velasco M, Borra Cebrian C, Panozzo C, Frugier T, et al. Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model. Hum Mol Genet. 2002;11:1439-47 pubmed
    ..Smn mutant mice should represent a valuable model for elucidating the pathway linking Smn to cytoskeletal organization. ..
  8. Engel A, Ohno K, Bouzat C, Sine S, Griggs R. End-plate acetylcholine receptor deficiency due to nonsense mutations in the epsilon subunit. Ann Neurol. 1996;40:810-7 pubmed
    ..Expression of gamma-AChR at the EPs may serve as the means of phenotypic rescue from potentially fatal nonsense mutations in the epsilon-subunit gene. ..
  9. Smith R, Chung H, Rundquist S, Maat Schieman M, Colgan L, Englund E, et al. Cholinergic neuronal defect without cell loss in Huntington's disease. Hum Mol Genet. 2006;15:3119-31 pubmed
    ..Taken together, our data show that the cholinergic system is dysfunctional in R6/1 and HD patients. Consequently, they provide a rationale for testing of pro-cholinergic drugs in this disease. ..

More Information

Publications62

  1. Amirali A, Mu L, Gracies J, Simpson D. Anatomical localization of motor endplate bands in the human biceps brachii. J Clin Neuromuscul Dis. 2007;9:306-12 pubmed
    ..This study localized the motor endplate bands (MEB) within human biceps brachii (BB) muscles in relation to external landmarks...
  2. Kohrman D, Plummer N, Schuster T, Jones J, Jang W, Burgess D, et al. Insertional mutation of the motor endplate disease (med) locus on mouse chromosome 15. Genomics. 1995;26:171-7 pubmed
    ..The transgene insertion site was mapped to distal chromosome 15 close to the locus motor endplate disease (med). The sequence of mouse DNA flanking the insertion site junctions was determined...
  3. Angaut Petit D, McArdle J, Mallart A, Bournaud R, Pincon Raymond M, Rieger F. Electrophysiological and morphological studies of a motor nerve in 'motor endplate disease' of the mouse. Proc R Soc Lond B Biol Sci. 1982;215:117-25 pubmed
    Motor nerves of mice affected with hereditary 'motor endplate disease' were examined by means of electrophysiological and morphological techniques...
  4. Ohno K, Hutchinson D, Milone M, Brengman J, Bouzat C, Sine S, et al. Congenital myasthenic syndrome caused by prolonged acetylcholine receptor channel openings due to a mutation in the M2 domain of the epsilon subunit. Proc Natl Acad Sci U S A. 1995;92:758-62 pubmed
    ..This observation raises the possibility that mutations involving subunits of other ligand-gated channels may also exist and be the basis of various other neurologic or psychiatric disorders. ..
  5. Uchitel O, Engel A, Walls T, Nagel A, Atassi M, Bril V. Congenital myasthenic syndromes: II. Syndrome attributed to abnormal interaction of acetylcholine with its receptor. Muscle Nerve. 1993;16:1293-301 pubmed
  6. Burgess D, Kohrman D, Galt J, Plummer N, Jones J, Spear B, et al. Mutation of a new sodium channel gene, Scn8a, in the mouse mutant 'motor endplate disease'. Nat Genet. 1995;10:461-5 pubmed
    The mouse neurological mutant 'motor endplate disease' (med) is characterized by early onset progressive paralysis of the hind limbs, severe muscle atrophy, degeneration of Purkinje cells and juvenile lethality...
  7. Rigoard P, Buffenoir K, Bauche S, Giot J, Koenig J, Hantai D, et al. [Structural and molecular organization, development and maturation of the neuromuscular junction]. Neurochirurgie. 2009;55 Suppl 1:S34-42 pubmed publisher
    ..The initial steps of this differentiation and the motor endplate formation require several postsynaptic molecular agents including agrin, the tyrosine kinase receptor MuSK...
  8. Tóth A, Szucs A, Harasztosi C, Matesz K, Pucsok K, Miko I, et al. Intrinsic laryngeal muscle reinnervation with nerve-muscle pedicle. Otolaryngol Head Neck Surg. 2005;132:701-6 pubmed
    ..To test the application of the nerve-muscle pedicle (NMP) technique for selective reinnervation of previously denervated posterior cricoarytenoid (PCA) muscle...
  9. Liewluck T, Shen X, Milone M, Engel A. Endplate structure and parameters of neuromuscular transmission in sporadic centronuclear myopathy associated with myasthenia. Neuromuscul Disord. 2011;21:387-95 pubmed publisher
    ..The safety margin of neuromuscular transmission is compromised by decreased quantal release by nerve impulse and by a reduced postsynaptic response to the released quanta...
  10. Angelov D, Ceynowa M, Guntinas Lichius O, Streppel M, Grosheva M, Kiryakova S, et al. Mechanical stimulation of paralyzed vibrissal muscles following facial nerve injury in adult rat promotes full recovery of whisking. Neurobiol Dis. 2007;26:229-42 pubmed
    ..Our findings indicate the potential of use-specific training to enhance appropriate functional outcome after peripheral nerve injury and may be useful in a clinical rehabilitation setting. ..
  11. Santafe M, Salon I, Garcia N, Lanuza M, Uchitel O, Tomas J. Modulation of ACh release by presynaptic muscarinic autoreceptors in the neuromuscular junction of the newborn and adult rat. Eur J Neurosci. 2003;17:119-27 pubmed
    ..We show that the way in which M1 and M2 muscarinic receptors modulate neurotransmission can differ between the developing and adult rat neuromuscular synapse. ..
  12. Jin T, Wernig A, Witzemann V. Changes in acetylcholine receptor function induce shifts in muscle fiber type composition. FEBS J. 2008;275:2042-54 pubmed publisher
    ..Muscle thus represents a self-matching system that adjusts contractile properties and synaptic function to variable functional demands...
  13. Giniatullin A, Darios F, Shakirzyanova A, Davletov B, Giniatullin R. SNAP25 is a pre-synaptic target for the depressant action of reactive oxygen species on transmitter release. J Neurochem. 2006;98:1789-97 pubmed
    ..This mechanism of ROS signalling could have widespread implications in the nervous system and might contribute to the pathogenesis of neurodegenerative diseases. ..
  14. Sine S. End-plate acetylcholine receptor: structure, mechanism, pharmacology, and disease. Physiol Rev. 2012;92:1189-234 pubmed publisher
    ..receptors increased at a rapid pace, owing largely to studies of the acetylcholine receptor (AChR) at the motor endplate. The endplate AChR belongs to a large superfamily of neurotransmitter receptors, called Cys-loop receptors, ..
  15. Mann M, Das S, Zhang J, Wagner M, Fischbach G. Neuregulin effect on quantal content dissociated from effect on miniature endplate potential amplitude. J Neurophysiol. 2006;96:671-6 pubmed
    ..Thus, during postnatal maturation, both quantal size and quantum content were influenced by decreased Ig-NRG expression, although the effects were dissociated in time. ..
  16. Bahia El Idrissi N, Bosch S, Ramaglia V, Aronica E, Baas F, Troost D. Complement activation at the motor end-plates in amyotrophic lateral sclerosis. J Neuroinflammation. 2016;13:72 pubmed publisher
    ..This study adds to the understanding of ALS pathology in man and identifies complement as a potential modifier of the disease process. ..
  17. Wu Y, Collier L, Qin W, Creasey G, Bauman W, Jarvis J, et al. Electrical stimulation modulates Wnt signaling and regulates genes for the motor endplate and calcium binding in muscle of rats with spinal cord transection. BMC Neurosci. 2013;14:81 pubmed publisher
    ..Notch and Wnt signaling may be involved in ES-induced increases in the mass of paralyzed muscle. Molecular adaptations of paralyzed soleus to resistance exercise are delayed or defective compared to normally innervated muscle. ..
  18. Mito K, Sakamoto K. On the evaluation of muscle fiber conduction velocity considering waveform properties of an electromyogram in M. biceps brachii during voluntary isometric contraction. Electromyogr Clin Neurophysiol. 2002;42:137-49 pubmed
    ..e., the regions other than the motor end-plate zone and the tendon zone. A high maximum correlation coefficient and a high amplitude ratio were necessary for a reliable measurement of the MFCV. ..
  19. Ishigaki K, Nicolle D, Krejci E, Leroy J, Koenig J, Fardeau M, et al. Two novel mutations in the COLQ gene cause endplate acetylcholinesterase deficiency. Neuromuscul Disord. 2003;13:236-44 pubmed
    ..The close similarity of the mutations of these two patients with different phenotypes suggests that other factors may modify the severity of this disease. ..
  20. Rafuse V, Polo Parada L, Landmesser L. Structural and functional alterations of neuromuscular junctions in NCAM-deficient mice. J Neurosci. 2000;20:6529-39 pubmed
  21. Sakamoto K, Mito K. Muscle fiber conduction velocity during isometric contraction and the recovery period. Electromyogr Clin Neurophysiol. 2000;40:151-61 pubmed
    ..Changes of the MFCV during the isometric contraction and the recovery period depend greatly on the location of the electrodes measured for the action potentials of the muscle fibers. ..
  22. Banwell B, Ohno K, Sieb J, Engel A. Novel truncating RAPSN mutations causing congenital myasthenic syndrome responsive to 3,4-diaminopyridine. Neuromuscul Disord. 2004;14:202-7 pubmed
  23. Kohara N, Lin T, Fukudome T, Kimura J, Sakamoto T, Kaji R, et al. Pathophysiology of weakness in a patient with congenital end-plate acetylcholinesterase deficiency. Muscle Nerve. 2002;25:585-92 pubmed
    ..These characteristics may constitute the primary underlying pathophysiologic mechanism in our patient and in similar cases of congenital myasthenic syndrome. ..
  24. Timóteo M, Oliveira L, Campesatto Mella E, Barroso A, Silva C, Magalhães Cardoso M, et al. Tuning adenosine A1 and A2A receptors activation mediates L-citrulline-induced inhibition of [3H]-acetylcholine release depending on nerve stimulation pattern. Neurochem Int. 2008;52:834-45 pubmed
    ..While adenosine acts predominantly at inhibitory A(1) receptors during 5 Hz-trains, inhibition of ACh release by L-citrulline at 50 Hz-bursts depends on the interplay between adenosine A(2A) and muscarinic M(2) receptors. ..
  25. Kuan T, Chen J, Chen S, Chien C, Hong C. Effect of botulinum toxin on endplate noise in myofascial trigger spots of rabbit skeletal muscle. Am J Phys Med Rehabil. 2002;81:512-20; quiz 521-3 pubmed
    ..The prevalence of endplate noise in the myofascial trigger point region may be a useful objective indicator for evaluating the therapeutic effectiveness of BTX-A injection to treat myofascial trigger points. ..
  26. Edwards J, Hatton P, Wareham A. Electrophysiology of the neuromuscular junction of the laminin-2 (merosin) deficient C57 BL/6J dy2J/dy2J dystrophic mouse. Brain Res. 1998;788:262-8 pubmed
    ..Some changes appear from a direct effect of dystrophy, whilst increased MEPP frequency and quantal content, and failures in neurotransmission indicate neuronal abnormalities. ..
  27. Zefirov A, Abdrakhmanov M, Grigor ev P. [Effects of high potassium solutions and caffeine on the processes of exo-endocytosis of synaptic vesicles at the frog motor nerve ending]. Ross Fiziol Zh Im I M Sechenova. 2005;91:821-31 pubmed
    ..Possible spatial localization of endoplasmic reticulum at the motor nerve ending is discussed. The hypothesis of its role at the remodeling of synapse was proposed. ..
  28. Vega Riveroll L, Wylie S, Loughna P, Parson S, Chantler P. Nonmuscle myosins IIA and IIB are present in adult motor nerve terminals. Neuroreport. 2005;16:1143-6 pubmed
    ..We propose roles for these motor proteins in junctional stabilization and destabilization. ..
  29. Papakonstantinou K, Kamin E, Terzis J. Muscle preservation by prolonged sensory protection. J Reconstr Microsurg. 2002;18:173-82; discussion 183-4 pubmed
    ..Evaluation also included intraoperative electrical stimulation of the MC nerve, biceps muscle dry weights, motor endplate counts, and nerve axon counts of the MC nerve...
  30. Greising S, Stowe J, Sieck G, Mantilla C. Role of TrkB kinase activity in aging diaphragm neuromuscular junctions. Exp Gerontol. 2015;72:184-91 pubmed publisher
    ..Collectively these results support a period of susceptibility in early old age when BDNF/TrkB signaling at diaphragm NMJs supports the maintenance of NMJs structure and muscle innervation. ..
  31. Ruff R, Lennon V. How myasthenia gravis alters the safety factor for neuromuscular transmission. J Neuroimmunol. 2008;201-202:13-20 pubmed publisher
    ..We conclude that loss of endplate Na(+) channels in MG is due to complement-mediated loss of endplate membrane rather than a direct effect of myasthenogenic antibodies on endplate Na(+) channels. ..
  32. Hsieh Y, Chou L, Joe Y, Hong C. Spinal cord mechanism involving the remote effects of dry needling on the irritability of myofascial trigger spots in rabbit skeletal muscle. Arch Phys Med Rehabil. 2011;92:1098-105 pubmed publisher
    ..This remote effect of dry needling depends on an intact afferent pathway from the stimulating site to the spinal cord and a normal spinal cord function at the levels corresponding to the innervation of the proximally affected muscle. ..
  33. Cousin M. Vulpian and not Claude Bernard first proposed the hypothesis of the motor end-plate as the site of action of curare. Anesthesiology. 2002;97:527-8 pubmed
  34. Vohra B, Groshong J, Maselli R, Verity M, Wollmann R, Gomez C. Focal caspase activation underlies the endplate myopathy in slow-channel syndrome. Ann Neurol. 2004;55:347-52 pubmed
    ..These findings provide the first evidence supporting the view that caspase activation in human disease can play a prominent role in localized cellular degenerative processes without causing nuclear or cell death. ..
  35. Malomuzh A, Mukhtarov M, Urazaev A, Nikol skiĭ E, Vyskocil F. [Effect of glutamate on spontaneous secretion of acetylcholine in the nerve-muscle synapse in rats]. Ross Fiziol Zh Im I M Sechenova. 2001;87:492-8 pubmed
    ..Glutamate seems to modulate the nonquantal acetylcholine secretion by initiation of the NO synthesis in muscle fibres via activation of the NMDA receptors. ..
  36. Milone M, Fukuda T, Shen X, Tsujino A, Brengman J, Engel A. Novel congenital myasthenic syndromes associated with defects in quantal release. Neurology. 2006;66:1223-9 pubmed
    ..Combined clinical, morphologic, and in vitro electrophysiologic findings define two novel congenital myasthenic syndromes. The molecular basis of these syndromes awaits discovery. ..
  37. Tosolini A, Morris R. Spatial characterization of the motor neuron columns supplying the rat forelimb. Neuroscience. 2012;200:19-30 pubmed publisher
    ..The length of these columns, and hence the overlap between them, appears to be greater than previously reported, particularly within the uppermost segments of the brachial plexus. ..
  38. Bowen D, Park J, Bodine S, Stark J, Valenzuela D, Stitt T, et al. Localization and regulation of MuSK at the neuromuscular junction. Dev Biol. 1998;199:309-19 pubmed
    ..Here we show that MuSK is associated with the earliest observable AChR clusters at the developing motor endplate and that MuSK and AChRs codistribute throughout the development of the NMJ...
  39. Orhan E, Deymeer F, Oflazer P, Parman Y, Baslo M. Jitter analysis with concentric needle electrode in the masseter muscle for the diagnosis of generalised myasthenia gravis. Clin Neurophysiol. 2013;124:2277-82 pubmed publisher
    ..Jitter analysis of the masseter muscle during voluntary contraction is easy to perform and it was found as informative as other muscles in patients with generalised MG. ..
  40. Heier T. [Muscle relaxants]. Tidsskr Nor Laegeforen. 2010;130:398-401 pubmed publisher
    ..The purpose of this article is to provide an overview of the history of muscle relaxants, their mode of action and their role in current anaesthetic practice...
  41. Harris A, Duxson M, Butler J, Hodges P, Taylor J, Gandevia S. Muscle fiber and motor unit behavior in the longest human skeletal muscle. J Neurosci. 2005;25:8528-33 pubmed
  42. Cossins J, Webster R, Maxwell S, Burke G, Vincent A, Beeson D. A mouse model of AChR deficiency syndrome with a phenotype reflecting the human condition. Hum Mol Genet. 2004;13:2947-57 pubmed
    ..They display fatigable muscle weakness, reduced miniature endplate potentials and endplate potentials, reduced motor endplate AChR number and altered endplate morphology...
  43. Bischoff A, Grosheva M, Irintchev A, Skouras E, Kaidoglou K, Michael J, et al. Manual stimulation of the orbicularis oculi muscle improves eyelid closure after facial nerve injury in adult rats. Muscle Nerve. 2009;39:197-205 pubmed publisher
    ..42 +/- 10% without manual stimulation, 0% in intact rats). We conclude that manual stimulation is a noninvasive and simple procedure with immediate potential for clinical rehabilitation of eyelid closure following facial nerve injury. ..
  44. Gramsbergen A, Ijkema Paassen J, Nikkels P, Hadders Algra M. Regression of polyneural innervation in the human psoas muscle. Early Hum Dev. 1997;49:49-61 pubmed
    ..Motor endplates were stained by a combined acetylcholinesterase stain. Innervation patterns and motor endplate morphology were studied and the sizes of endplates were measured...
  45. Gomez C, Maselli R, Groshong J, Zayas R, Wollmann R, Cens T, et al. Active calcium accumulation underlies severe weakness in a panel of mice with slow-channel syndrome. J Neurosci. 2002;22:6447-57 pubmed
    ..Finally, severe degenerative changes at the motor endplate (endplate myopathy) were apparent by electron microscopy in transgenic lines that displayed the greatest ..
  46. Moravec J, Vyskocil F. Early postdenervation depolarization develops faster at endplates of hibernating golden hamsters where spontaneous quantal and non-quantal acetylcholine release is very small. Neurosci Res. 2005;51:25-9 pubmed
    ..Savchenko, V., Apparsundaram, S., Zwick, M., Wright J., Heilman, C.J., Yi, H., Levey, A.I., Blakely R.D. Vesicular localization and activity-dependent trafficking of presynaptic choline transporters. J. Neurosci. 23 (2003) 9697-9709]. ..
  47. Bukharaeva E, Samigullin D, Nikol skiĭ E, Vyskochil F. The role of intracellular cAMP in mediating the synchronizing action of noradrenaline on the evoked release of quanta of mediator in the frog synapse. Neurosci Behav Physiol. 2001;31:473-80 pubmed
    ..It was concluded that the action of noradrenaline on the time course of secretion is mediated by activation of presynaptic beta receptors, increased adenylate cyclase activity, and increases in intracellular cAMP levels. ..
  48. Wang X, Engisch K, Li Y, Pinter M, Cope T, Rich M. Decreased synaptic activity shifts the calcium dependence of release at the mammalian neuromuscular junction in vivo. J Neurosci. 2004;24:10687-92 pubmed
    ..Block of presynaptic P/Q channels eliminated the increase in probability of release. We propose that activity-dependent regulation of presynaptic calcium entry may contribute to homeostatic regulation of quantal content. ..
  49. Paez Colasante X, Seaberg B, Martinez T, Kong L, Sumner C, Rimer M. Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons. PLoS ONE. 2013;8:e75866 pubmed publisher
    ..Our results directly suggest that even those therapeutic interventions with very modest effects in raising SMN in motor neurons may provide mitigation of neuromuscular phenotypes in SMA patients. ..
  50. Sieck G, van Balkom R, Prakash Y, Zhan W, Dekhuijzen P. Corticosteroid effects on diaphragm neuromuscular junctions. J Appl Physiol (1985). 1999;86:114-22 pubmed
    ..direct muscle stimulation, was less in CS-treated Dimus. These results indicate that alterations in NMJ morphology after CS treatment are dependent on fiber type and may contribute to improved neuromuscular transmission...
  51. Samigullin D, Bukharaeva E, Nikolsky E, Adamek S, Vyskocil F. Long release latencies are increased by acetylcholine at frog endplate. Physiol Res. 2003;52:475-80 pubmed
    ..This indicates that the evoked release pattern is less synchronous and the increased asynchrony leads to a substantial drop (by 28 per cent) in the amplitude of reconstructed multi-quantal currents...
  52. Dimitrova N, Disselhorst Klug C, Dimitrov G, Rau G. Area-to-amplitude ratio of the terminal phase of the belly-tendon detected motor unit potentials could be used to recognize reinnervated motor units. Electromyogr Clin Neurophysiol. 2002;42:267-73 pubmed
    ..Basing on the results obtained, we can conclude that the ratio reflects main functional compensations in reinnervated MUs and could be used for discrimination between reinnervated and normal MUs. ..
  53. Nekkanty S, Yerramshetty J, Kim D, Zauel R, Johnson E, Cody D, et al. Stiffness of the endplate boundary layer and endplate surface topography are associated with brittleness of human whole vertebral bodies. Bone. 2010;47:783-9 pubmed publisher
    ..Our results indicate that endplate topography variations may provide insight into the mechanisms responsible for vertebral fractures. ..