Experts and Doctors on motor neurons in California, United States


Locale: California, United States
Topic: motor neurons

Top Publications

  1. Yu F, Sugawara T, Nishi T, Liu J, Chan P. Overexpression of SOD1 in transgenic rats attenuates nuclear translocation of endonuclease G and apoptosis after spinal cord injury. J Neurotrauma. 2006;23:595-603 pubmed
    ..An increased level of SOD1 in mitochondria conceivably reduces oxidative stress, thereby attenuating EndoG translocation, and resulting in reduction of caspase-independent apoptosis. ..
  2. Rao M, Houseweart M, Williamson T, Crawford T, Folmer J, Cleveland D. Neurofilament-dependent radial growth of motor axons and axonal organization of neurofilaments does not require the neurofilament heavy subunit (NF-H) or its phosphorylation. J Cell Biol. 1998;143:171-81 pubmed
  3. Lai Y, Kodama T, Siegel J. Changes in monoamine release in the ventral horn and hypoglossal nucleus linked to pontine inhibition of muscle tone: an in vivo microdialysis study. J Neurosci. 2001;21:7384-91 pubmed
  4. Song M. Moving cell bodies: understanding the migratory mechanism of facial motor neurons. Arch Pharm Res. 2007;30:1273-82 pubmed
  5. Martin P, Scott L, Porter B, Sanes J. Distinct structures and functions of related pre- and postsynaptic carbohydrates at the mammalian neuromuscular junction. Mol Cell Neurosci. 1999;13:105-18 pubmed
    ..Together, these results show that pre- as well as postsynaptic membranes bear and are affected by distinct but related synapse-specific carbohydrates. ..
  6. Nguyen Q, Kleinfeld D. Positive feedback in a brainstem tactile sensorimotor loop. Neuron. 2005;45:447-57 pubmed
    ..On the basis of these findings, we propose that the trigeminal loop provides an enhancement of vibrissa muscle tone upon contact during active touch. ..
  7. Cosman M, Lightstone F, Krishnan V, Zeller L, Prieto M, Roe D, et al. Identification of novel small molecules that bind to two different sites on the surface of tetanus toxin C fragment. Chem Res Toxicol. 2002;15:1218-28 pubmed
  8. Schwieterman A, Steves A, Yee V, Donelson C, Bentley M, Santorella E, et al. The Caenorhabditis elegans Ephrin EFN-4 Functions Non-cell Autonomously with Heparan Sulfate Proteoglycans to Promote Axon Outgrowth and Branching. Genetics. 2016;202:639-60 pubmed publisher
    ..This is the first report of an epidermal ephrin providing a developmental cue to the nervous system. ..
  9. Llewellyn M, Thompson K, Deisseroth K, Delp S. Orderly recruitment of motor units under optical control in vivo. Nat Med. 2010;16:1161-5 pubmed publisher
    ..These findings point to an unanticipated new modality of neural control with broad implications for nervous system and neuromuscular physiology, disease research and therapeutic innovation. ..

More Information


  1. Cleveland D, Rothstein J. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci. 2001;2:806-19 pubmed
  2. Philips G, Sherff C, Menges S, Carew T. The tail-elicited tail withdrawal reflex of Aplysia is mediated centrally at tail sensory-motor synapses and exhibits sensitization across multiple temporal domains. Learn Mem. 2011;18:272-82 pubmed publisher
    ..This preparation now provides an experimentally powerful system in which to directly examine the unique and combined roles of synaptic and nuclear signaling in different temporal domains of memory formation...
  3. Gallarda B, Sharpee T, Pfaff S, Alaynick W. Defining rhythmic locomotor burst patterns using a continuous wavelet transform. Ann N Y Acad Sci. 2010;1198:133-9 pubmed publisher
    ..The CWT provides greater resolution of cycle period, phase, and rhythmicity, and is proving to be a superior technique in assessing subtle changes in locomotion due to genetic perturbations of the underlying circuitry. ..
  4. Houseweart M, Cleveland D. Bcl-2 overexpression does not protect neurons from mutant neurofilament-mediated motor neuron degeneration. J Neurosci. 1999;19:6446-56 pubmed
  5. Liu S, Sawada T, Lee S, Yu W, Silverio G, Alapatt P, et al. Parkinson's disease-associated kinase PINK1 regulates Miro protein level and axonal transport of mitochondria. PLoS Genet. 2012;8:e1002537 pubmed publisher
    ..Moreover, the different effects of loss of PINK1 function on Miro protein level in Drosophila and mouse cells may offer one explanation of the distinct phenotypic manifestations of PINK1 mutants in these two species. ..
  6. Goulding M. Circuits controlling vertebrate locomotion: moving in a new direction. Nat Rev Neurosci. 2009;10:507-18 pubmed publisher
  7. Patel M, Lehrman E, Poon V, Crump J, Zhen M, Bargmann C, et al. Hierarchical assembly of presynaptic components in defined C. elegans synapses. Nat Neurosci. 2006;9:1488-98 pubmed
  8. Shirasaki R, Lewcock J, Lettieri K, Pfaff S. FGF as a target-derived chemoattractant for developing motor axons genetically programmed by the LIM code. Neuron. 2006;50:841-53 pubmed
    ..These results point to a role for FGF signaling in axon guidance and further unravel how downstream effectors of LIM codes direct wiring of the developing nervous system. ..
  9. Jeon M, Nguyen H, Bahri S, Zinn K. Redundancy and compensation in axon guidance: genetic analysis of the Drosophila Ptp10D/Ptp4E receptor tyrosine phosphatase subfamily. Neural Dev. 2008;3:3 pubmed publisher
    ..Strong phenotypes are only observed in triple mutants in which both type III RPTPs are eliminated together with Ptp69D or Ptp52F. Our results allow us to construct a complete genetic interaction matrix for all six of the RPTPs. ..
  10. Yu F, Narasimhan P, Saito A, Liu J, Chan P. Increased expression of a proline-rich Akt substrate (PRAS40) in human copper/zinc-superoxide dismutase transgenic rats protects motor neurons from death after spinal cord injury. J Cereb Blood Flow Metab. 2008;28:44-52 pubmed
  11. Parone P, Da Cruz S, Han J, McAlonis Downes M, Vetto A, Lee S, et al. Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci. 2013;33:4657-71 pubmed publisher
  12. Marquardt T, Shirasaki R, Ghosh S, Andrews S, Carter N, Hunter T, et al. Coexpressed EphA receptors and ephrin-A ligands mediate opposing actions on growth cone navigation from distinct membrane domains. Cell. 2005;121:127-39 pubmed
  13. Myers C, Lewcock J, Hanson M, Gosgnach S, Aimone J, Gage F, et al. Cholinergic input is required during embryonic development to mediate proper assembly of spinal locomotor circuits. Neuron. 2005;46:37-49 pubmed
    ..These findings suggest that the cholinergic neurotransmitter pathway is involved in configuring the CPG during a transient period of development. ..
  14. Lagier Tourenne C, Polymenidou M, Hutt K, Vu A, Baughn M, Huelga S, et al. Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci. 2012;15:1488-97 pubmed publisher
  15. Spilker K, Wang G, Tugizova M, Shen K. Caenorhabditis elegans Muscleblind homolog mbl-1 functions in neurons to regulate synapse formation. Neural Dev. 2012;7:7 pubmed publisher
    ..Consistent with this result, mbl-1 is also expressed in neurons. Based on these results, we conclude that in addition to its functions in muscle, the Muscleblind splice regulators also function in neurons to regulate synapse formation. ..
  16. Towne C, Montgomery K, Iyer S, Deisseroth K, Delp S. Optogenetic control of targeted peripheral axons in freely moving animals. PLoS ONE. 2013;8:e72691 pubmed publisher
    ..These methods can be broadly applied to study PNS disorders and lay the groundwork for future therapeutic application of optogenetics. ..
  17. Maro G, Klassen M, Shen K. A beta-catenin-dependent Wnt pathway mediates anteroposterior axon guidance in C. elegans motor neurons. PLoS ONE. 2009;4:e4690 pubmed publisher
    ..Together, our data provide evidence that Wnt-mediated axon guidance can be transduced through a beta-catenin-dependent pathway. ..
  18. Berdeaux R, Goebel N, Banaszynski L, Takemori H, WANDLESS T, Shelton G, et al. SIK1 is a class II HDAC kinase that promotes survival of skeletal myocytes. Nat Med. 2007;13:597-603 pubmed
  19. Sharma K, Leonard A, Lettieri K, Pfaff S. Genetic and epigenetic mechanisms contribute to motor neuron pathfinding. Nature. 2000;406:515-9 pubmed
    ..Nevertheless, elevated occupancy of the axial pathway can override their genetic program, causing some axons to project to alternative targets. ..
  20. 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. ..
  21. Sharma K, Sheng H, Lettieri K, Li H, Karavanov A, Potter S, et al. LIM homeodomain factors Lhx3 and Lhx4 assign subtype identities for motor neurons. Cell. 1998;95:817-28 pubmed
    ..Thus, Lhx3 and Lhx4 act in a binary fashion during a brief period in development to specify the trajectory of motor axons from the neural tube. ..
  22. Sapir T, Geiman E, Wang Z, Velasquez T, Mitsui S, Yoshihara Y, et al. Pax6 and engrailed 1 regulate two distinct aspects of renshaw cell development. J Neurosci. 2004;24:1255-64 pubmed
    ..Together, these results show that Pax6 and En1 have essential roles in establishing the recurrent inhibitory circuit between motor neurons and Renshaw cells. ..
  23. Blesch A, Tuszynski M. Cellular GDNF delivery promotes growth of motor and dorsal column sensory axons after partial and complete spinal cord transections and induces remyelination. J Comp Neurol. 2003;467:403-17 pubmed
    ..Thus, GDNF exerts tropic effects on adult spinal axons and Schwann cells that contribute to axon growth after injury. ..
  24. Stawicki T, Takayanagi Kiya S, Zhou K, Jin Y. Neuropeptides function in a homeostatic manner to modulate excitation-inhibition imbalance in C. elegans. PLoS Genet. 2013;9:e1003472 pubmed publisher
  25. Levine A, Hinckley C, Hilde K, Driscoll S, Poon T, Montgomery J, et al. Identification of a cellular node for motor control pathways. Nat Neurosci. 2014;17:586-93 pubmed publisher
    ..We speculate that these neurons comprise a cellular network for encoding coordinated motor output programs. ..
  26. Chia P, Patel M, Shen K. NAB-1 instructs synapse assembly by linking adhesion molecules and F-actin to active zone proteins. Nat Neurosci. 2012;15:234-42 pubmed publisher
    ..Altogether, we identify a role for the actin cytoskeleton during presynaptic development and characterize a molecular pathway whereby NAB-1 links synaptic partner recognition to active zone assembly. ..
  27. Rupp F, Payan D, Magill Solc C, Cowan D, Scheller R. Structure and expression of a rat agrin. Neuron. 1991;6:811-23 pubmed
    ..The agrin gene is expressed in rat embryonic nervous system and muscle. The rat agrin protein is concentrated at synapses, where it may play a role in development and regeneration. ..
  28. Jurata L, Pfaff S, Gill G. The nuclear LIM domain interactor NLI mediates homo- and heterodimerization of LIM domain transcription factors. J Biol Chem. 1998;273:3152-7 pubmed
    ..Evidence indicating that NLI facilitates long range enhancer-promoter interactions suggests that NLI-dependent LIM domain transcription factor complexes are involved in communication between transcriptional control elements. ..
  29. Sann S, Crane M, Lu H, Jin Y. Rabx-5 regulates RAB-5 early endosomal compartments and synaptic vesicles in C. elegans. PLoS ONE. 2012;7:e37930 pubmed publisher
    ..These results suggest that rabx-5 regulation of RAB-5 compartments is important for maintaining proper synaptic function throughout the lifetime...
  30. Brandon E, Lin W, D Amour K, Pizzo D, Dominguez B, Sugiura Y, et al. Aberrant patterning of neuromuscular synapses in choline acetyltransferase-deficient mice. J Neurosci. 2003;23:539-49 pubmed
    ..These results indicate that ChAT is uniquely essential for the patterning and formation of mammalian neuromuscular synapses. ..
  31. Thaler J, Harrison K, Sharma K, Lettieri K, Kehrl J, Pfaff S. Active suppression of interneuron programs within developing motor neurons revealed by analysis of homeodomain factor HB9. Neuron. 1999;23:675-87 pubmed
    ..Thus, MNs actively suppress interneuron genetic programs to establish their identity...
  32. Rahman A, Kamal A, Roberts E, Goldstein L. Defective kinesin heavy chain behavior in mouse kinesin light chain mutants. J Cell Biol. 1999;146:1277-88 pubmed
    ..Taken together, these data best support models that suggest that KLC1 is essential for proper KHC activation or targeting. ..
  33. Kilpatrick T, Brown A, Lai C, Gassmann M, Goulding M, Lemke G. Expression of the Tyro4/Mek4/Cek4 gene specifically marks a subset of embryonic motor neurons and their muscle targets. Mol Cell Neurosci. 1996;7:62-74 pubmed
    ..Mek4 has previously been ascribed a role in guiding retinal axons to their targets in the optic tectum. Our results extend the purported role of Mek4 in axon guidance to include motor neurons of the medial motor column. ..
  34. Gosgnach S, Lanuza G, Butt S, Saueressig H, Zhang Y, Velasquez T, et al. V1 spinal neurons regulate the speed of vertebrate locomotor outputs. Nature. 2006;440:215-9 pubmed
  35. Chesi A, Staahl B, Jovicic A, Couthouis J, Fasolino M, Raphael A, et al. Exome sequencing to identify de novo mutations in sporadic ALS trios. Nat Neurosci. 2013;16:851-5 pubmed publisher
    ..These findings expand our understanding of the ALS genetic landscape and provide a resource for future studies into the pathogenic mechanisms contributing to sporadic ALS. ..
  36. Kim H, Raphael A, LADOW E, McGurk L, Weber R, Trojanowski J, et al. Therapeutic modulation of eIF2? phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models. Nat Genet. 2014;46:152-60 pubmed publisher
    ..These findings indicate that the dysfunction induced by prolonged stress granule formation might contribute directly to ALS and that compounds that mitigate this process may represent a novel therapeutic approach. ..
  37. Lin W, Burgess R, Dominguez B, Pfaff S, Sanes J, Lee K. Distinct roles of nerve and muscle in postsynaptic differentiation of the neuromuscular synapse. Nature. 2001;410:1057-64 pubmed
    ..In contrast, the subsequent stages of synaptic growth and maintenance require nerve-derived agrin, and a second nerve-derived signal that disperses ectopic postsynaptic apparatus. ..
  38. Morris J, Lin W, Hauser C, Marchuk Y, Getman D, Lee K. Rescue of the cardiac defect in ErbB2 mutant mice reveals essential roles of ErbB2 in peripheral nervous system development. Neuron. 1999;23:273-83 pubmed
    ..However, these clusters are dispersed and morphologically different from those in control muscle. Our results reveal an important role for erbB2 during normal peripheral nervous system development. ..