motor neurons


Summary: Neurons which activate MUSCLE CELLS.

Top Publications

  1. Mitchell J, McGoldrick P, Vance C, Hortobagyi T, Sreedharan J, Rogelj B, et al. Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Acta Neuropathol. 2013;125:273-88 pubmed publisher
    ..Large motor neurons were lost from the spinal cord accompanied by neurophysiological evidence of denervation and focal muscle ..
  2. Wu L, Cheng W, Shen C. Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice. J Biol Chem. 2012;287:27335-44 pubmed publisher
    ..mouse gene targeting approach, we show that mice with inactivation of the Tardbp gene in the spinal cord motor neurons (HB9:Cre-Tardbp(lx/-)) exhibit progressive and male-dominant development of ALS-related phenotypes including ..
  3. 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
    ..after TDP-43 or FUS/TLS depletion in stem cell-derived human neurons and in TDP-43 aggregate-containing motor neurons in sporadic ALS, supporting a common loss-of-function pathway as one component underlying motor neuron death ..
  4. Peters O, Millership S, Shelkovnikova T, Soto I, Keeling L, Hann A, et al. Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis. Neurobiol Dis. 2012;48:124-31 pubmed publisher
    ..previously associated with ALS, recapitulates key features of the disease, namely selective damage and loss of discrete populations of upper and lower motor neurons and their axons, contrasted by limited effects upon the sensory system.
  5. Frakes A, Ferraiuolo L, Haidet Phillips A, Schmelzer L, Braun L, Miranda C, et al. Microglia induce motor neuron death via the classical NF-?B pathway in amyotrophic lateral sclerosis. Neuron. 2014;81:1009-1023 pubmed publisher
  6. Meyer K, Ferraiuolo L, Miranda C, Likhite S, McElroy S, Renusch S, et al. Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A. 2014;111:829-32 pubmed publisher
    ..Non-cell autonomous toxicity to motor neurons is found following coculture of i-astrocytes from familial ALS patients with mutation in superoxide dismutase ..
  7. Lobsiger C, Boillée S, Pozniak C, Khan A, McAlonis Downes M, Lewcock J, et al. C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A. 2013;110:E4385-92 pubmed publisher
    ..component of the classic complement system that is the protein-based arm of the innate immune response, in motor neurons of multiple ALS mouse models expressing dismutase active or inactive SOD1 mutants...
  8. Sareen D, O Rourke J, Meera P, Muhammad A, Grant S, Simpkinson M, et al. Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med. 2013;5:208ra149 pubmed publisher
    Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition characterized by loss of motor neurons in the brain and spinal cord...
  9. Ma X, Turnbull P, Peterson R, Turnbull J. Trophic and proliferative effects of Shh on motor neurons in embryonic spinal cord culture from wildtype and G93A SOD1 mice. BMC Neurosci. 2013;14:119 pubmed publisher
    ..morphogen sonic hedgehog (Shh) may continue to play a trophic role in the support of terminally-differentiated motor neurons, of potential relevance to motor neuron disease...

More Information


  1. 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
    ..remained unclear if their short lifespan and motor weakness are primarily due to cell-autonomous defects in motor neurons. Using Hb9(Cre) as a driver, we selectively raised SMN expression in motor neurons in conditional SMA?7 mice...
  2. Han S, El Oussini H, Scekic Zahirovic J, Vibbert J, Cottee P, Prasain J, et al. VAPB/ALS8 MSP ligands regulate striated muscle energy metabolism critical for adult survival in caenorhabditis elegans. PLoS Genet. 2013;9:e1003738 pubmed publisher
    ..Abnormalities in energy metabolism of ALS patients may thus constitute a compensatory mechanism counterbalancing skeletal muscle mitochondrial dysfunction. ..
  3. Roberts B, Lim N, McAllum E, Donnelly P, Hare D, Doble P, et al. Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci. 2014;34:8021-31 pubmed publisher
    ..Improving the metal content of SOD1 therefore represents a valid therapeutic strategy for treating ALS caused by SOD1. ..
  4. Kaplan A, Spiller K, Towne C, Kanning K, Choe G, Geber A, et al. Neuronal matrix metalloproteinase-9 is a determinant of selective neurodegeneration. Neuron. 2014;81:333-48 pubmed publisher
    ..In patients with amyotrophic lateral sclerosis (ALS), most motor neurons die but those innervating extraocular, pelvic sphincter, and slow limb muscles exhibit selective resistance...
  5. Binan L, Tendey C, De Crescenzo G, El Ayoubi R, Ajji A, Jolicoeur M. Differentiation of neuronal stem cells into motor neurons using electrospun poly-L-lactic acid/gelatin scaffold. Biomaterials. 2014;35:664-74 pubmed publisher
    ..and without the instructive cues, differentiated into ?-III-tubulin, HB-9, Islet-1, and choactase-positive motor neurons by immunostaining, in response to the release of the biomolecules...
  6. Takahashi Y, Fukuda Y, Yoshimura J, Toyoda A, Kurppa K, Moritoyo H, et al. ERBB4 mutations that disrupt the neuregulin-ErbB4 pathway cause amyotrophic lateral sclerosis type 19. Am J Hum Genet. 2013;93:900-5 pubmed publisher
    Amyotrophic lateral sclerosis (ALS) is a devastating neurological disorder characterized by the degeneration of motor neurons and typically results in death within 3-5 years from onset...
  7. Saxena S, Roselli F, Singh K, Leptien K, Julien J, Gros Louis F, et al. Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival. Neuron. 2013;80:80-96 pubmed publisher
    ..Therefore, excitability and mTOR are key endogenous neuroprotection mechanisms in motoneurons to counteract clinically important disease progression in ALS. ..
  8. Wani K, Catanese M, Normantowicz R, Herd M, Maher K, Chase D. D1 dopamine receptor signaling is modulated by the R7 RGS protein EAT-16 and the R7 binding protein RSBP-1 in Caenoerhabditis elegans motor neurons. PLoS ONE. 2012;7:e37831 pubmed publisher
    ..Instead, EAT-16 and RSBP-1 act together to selectively inhibit D1 dopamine receptor signaling in cholinergic motor neurons to modulate locomotion behavior.
  9. Hideyama T, Teramoto S, Hachiga K, Yamashita T, Kwak S. Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons. PLoS ONE. 2012;7:e43469 pubmed publisher
    TDP-43 pathology in spinal motor neurons is a neuropathological hallmark of sporadic amyotrophic lateral sclerosis (ALS) and has recently been shown to be closely associated with the downregulation of an RNA editing enzyme called ..
  10. Ireland M, Funk G, Bellingham M. Muscarinic acetylcholine receptors enhance neonatal mouse hypoglossal motoneuron excitability in vitro. J Appl Physiol (1985). 2012;113:1024-39 pubmed publisher
  11. Berg A, Zelano J, Stephan A, Thams S, Barres B, Pekny M, et al. Reduced removal of synaptic terminals from axotomized spinal motoneurons in the absence of complement C3. Exp Neurol. 2012;237:8-17 pubmed publisher
    ..These data indicate that a non-classical pathway of complement activation is involved in axotomy-induced adult synapse removal, and that its inhibition promotes functional recovery. ..
  12. Wu H, Lu Y, Shen C, Patel N, Gan L, Xiong W, et al. Distinct roles of muscle and motoneuron LRP4 in neuromuscular junction formation. Neuron. 2012;75:94-107 pubmed publisher
    ..These observations uncovered distinct roles of LRP4 in motoneurons and muscles in NMJ development. ..
  13. Johnson C, Chun Jen Lin C, Stern M. Ras-dependent and Ras-independent effects of PI3K in Drosophila motor neurons. Genes Brain Behav. 2012;11:848-58 pubmed publisher
    ..if PI3K requires Ras activity for either of two different PI3K-regulated processes within Drosophila larval motor neurons. To address this question, we determined the effects on each process of transgenes and chromosomal mutations ..
  14. LaCombe J, Hanley O, Jung H, Philippidou P, Sürmeli G, Grinstein J, et al. Genetic and functional modularity of Hox activities in the specification of limb-innervating motor neurons. PLoS Genet. 2013;9:e1003184 pubmed publisher be preserved through a diverse array of Hox5-Hox8 paralogs, which are sufficient to reprogram thoracic motor neurons to an LMC fate...
  15. Hannemann M, Sasidharan N, Hegermann J, Kutscher L, Koenig S, Eimer S. TBC-8, a putative RAB-2 GAP, regulates dense core vesicle maturation in Caenorhabditis elegans. PLoS Genet. 2012;8:e1002722 pubmed publisher
    ..Therefore, we propose that a dynamic ON/OFF cycling of RAB-2 at the Golgi induced by the GAP/effector complex is required for proper DCV maturation. ..
  16. Audouard E, Schakman O, Rene F, Huettl R, Huber A, Loeffler J, et al. The Onecut transcription factor HNF-6 regulates in motor neurons the formation of the neuromuscular junctions. PLoS ONE. 2012;7:e50509 pubmed publisher
    The neuromuscular junctions are the specialized synapses whereby spinal motor neurons control the contraction of skeletal muscles...
  17. Wang L, Pytel P, Feltri M, Wrabetz L, Roos R. Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice. Neurobiol Dis. 2012;48:52-7 pubmed publisher
    ..The results suggest that mtSOD1-induced FALS treatment may have to be adjusted depending on the cell type targeted and particular mtSOD1 involved. ..
  18. Martinez T, Kong L, Wang X, Osborne M, Crowder M, Van Meerbeke J, et al. Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy. J Neurosci. 2012;32:8703-15 pubmed publisher
    ..To address whether this synaptic dysfunction is due to SMN deficiency in motor neurons, muscle, or both, we generated three lines of conditional SMA mice with tissue-specific increases in SMN ..
  19. Martin E, Cazenave W, Cattaert D, Branchereau P. Embryonic alteration of motoneuronal morphology induces hyperexcitability in the mouse model of amyotrophic lateral sclerosis. Neurobiol Dis. 2013;54:116-26 pubmed publisher
  20. Herron L, Miles G. Gender-specific perturbations in modulatory inputs to motoneurons in a mouse model of amyotrophic lateral sclerosis. Neuroscience. 2012;226:313-23 pubmed publisher
    ..In conclusion, these data support the involvement of pre-symptomatic changes in C-bouton anatomy in ALS pathogenesis and in particular mechanisms underlying the male bias of this disease. ..
  21. Skup M, Gajewska Wozniak O, Grygielewicz P, Mankovskaya T, Czarkowska Bauch J. Different effects of spinalization and locomotor training of spinal animals on cholinergic innervation of the soleus and tibialis anterior motoneurons. Eur J Neurosci. 2012;36:2679-88 pubmed publisher
  22. Rathod R, Havlicek S, Frank N, Blum R, Sendtner M. Laminin induced local axonal translation of ?-actin mRNA is impaired in SMN-deficient motoneurons. Histochem Cell Biol. 2012;138:737-48 pubmed publisher
    ..Taken together our findings suggest that local translation of ?-actin in growth cones of motoneurons is regulated by Laminin signalling and that this signalling is disturbed in SMA. ..
  23. Reimer M, Norris A, Ohnmacht J, Patani R, Zhong Z, Dias T, et al. Dopamine from the brain promotes spinal motor neuron generation during development and adult regeneration. Dev Cell. 2013;25:478-91 pubmed publisher
    ..successful spinal cord regeneration in adult zebrafish, endogenous dopamine promotes generation of spinal motor neurons, and dopamine agonists augment this process...
  24. Cherry J, Androphy E. Therapeutic strategies for the treatment of spinal muscular atrophy. Future Med Chem. 2012;4:1733-50 pubmed publisher
    ..muscular atrophy (SMA) is an inherited neurodegenerative disease that results in progressive dysfunction of motor neurons of the anterior horn of the spinal cord...
  25. Sun H, Knippenberg S, Thau N, Ragancokova D, Körner S, Huang D, et al. Therapeutic potential of N-acetyl-glucagon-like peptide-1 in primary motor neuron cultures derived from non-transgenic and SOD1-G93A ALS mice. Cell Mol Neurobiol. 2013;33:347-57 pubmed publisher
    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the death of motor neurons (MN) in the motor cortex, brain stem, and spinal cord...
  26. Kinoshita M, Matsui R, Kato S, Hasegawa T, Kasahara H, Isa K, et al. Genetic dissection of the circuit for hand dexterity in primates. Nature. 2012;487:235-8 pubmed publisher
    It is generally accepted that the direct connection from the motor cortex to spinal motor neurons is responsible for dexterous hand movements in primates...
  27. Dammer E, Fallini C, Gozal Y, Duong D, Rossoll W, Xu P, et al. Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination. PLoS ONE. 2012;7:e38658 pubmed publisher
    ..RRM-containing proteins co-enriched in cytoplasmic granular structures in HEK-293 cells and primary motor neurons with insoluble TDP-S6, including cytoplasmic stress granule associated proteins G3BP, PABPC1, and eIF4A1...
  28. Tsai H, Li H, Fuentealba L, Molofsky A, Taveira Marques R, Zhuang H, et al. Regional astrocyte allocation regulates CNS synaptogenesis and repair. Science. 2012;337:358-62 pubmed publisher
    ..Our findings demonstrate that region-restricted astrocyte allocation is a general CNS phenomenon and reveal intrinsic limitations of the astroglial response to injury. ..
  29. Fisher K, Zaaimi B, Williams T, Baker S, Baker M. Beta-band intermuscular coherence: a novel biomarker of upper motor neuron dysfunction in motor neuron disease. Brain. 2012;135:2849-64 pubmed publisher
    ..Therefore, intermuscular coherence has potential as a quantitative test of subclinical upper motor neuron involvement in motor neuron disease. ..
  30. 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
    ..lateral sclerosis (ALS) is a progressive, lethal neurodegenerative disorder characterised by selective loss of motor neurons with accompanying muscle paralysis and respiratory failure...
  31. Brockington A, Ning K, Heath P, Wood E, Kirby J, Fusi N, et al. Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity. Acta Neuropathol. 2013;125:95-109 pubmed publisher the sparing of eye movements and the function of external sphincters, with corresponding preservation of motor neurons in the brainstem oculomotor nuclei, and of Onuf's nucleus in the sacral spinal cord...
  32. Kallio J, Søgaard K, Avela J, Komi P, Selänne H, Linnamo V. Age-related decreases in motor unit discharge rate and force control during isometric plantar flexion. J Electromyogr Kinesiol. 2012;22:983-9 pubmed publisher
    ..These findings suggest that the age-related changes in MU control do exist also in large leg extensors that play an important role in human locomotion and balance control. ..
  33. Nicaise C, Putatunda R, Hala T, Regan K, Frank D, Brion J, et al. Degeneration of phrenic motor neurons induces long-term diaphragm deficits following mid-cervical spinal contusion in mice. J Neurotrauma. 2012;29:2748-60 pubmed publisher
    ..regions targets degeneration of both descending bulbospinal respiratory axons and cell bodies of phrenic motor neurons, resulting in deficits in the function of the diaphragm, the primary muscle of inspiration...
  34. Fallini C, Bassell G, Rossoll W. The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth. Hum Mol Genet. 2012;21:3703-18 pubmed publisher
    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease specifically affecting cortical and spinal motor neurons. Cytoplasmic inclusions containing hyperphosphorylated and ubiquitinated TDP-43 are a pathological hallmark of ..
  35. Lee A, Awano T, Park G, Monani U. Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy. PLoS ONE. 2012;7:e46353 pubmed publisher
    The selective vulnerability of motor neurons to paucity of Survival Motor Neuron (SMN) protein is a defining feature of human spinal muscular atrophy (SMA) and indicative of a unique requirement for adequate levels of the protein in ..
  36. Aliaga L, Lai C, Yu J, Chub N, Shim H, Sun L, et al. Amyotrophic lateral sclerosis-related VAPB P56S mutation differentially affects the function and survival of corticospinal and spinal motor neurons. Hum Mol Genet. 2013;22:4293-305 pubmed publisher
    ..VAPB protein was accumulated as large punctate in the soma and proximal dendrites of both corticospinal motor neurons (CSMNs) and spinal motor neurons (SMNs) in P56S VAPB Tg mice...
  37. Hao L, Duy P, Jontes J, WOLMAN M, Granato M, Beattie C. Temporal requirement for SMN in motoneuron development. Hum Mol Genet. 2013;22:2612-25 pubmed publisher
    ..These data support that SMN is needed early in development of motoneuron dendrites and axons to develop normally and that this is essential for proper connectivity and movement. ..
  38. Elliott K, Houston D, Fritzsch B. Transplantation of Xenopus laevis tissues to determine the ability of motor neurons to acquire a novel target. PLoS ONE. 2013;8:e55541 pubmed publisher
    ..At a cellular level this requires, for example, molecularly resolving how brainstem motor neurons change their innervation target from muscle fibers (branchial motor neurons) to neural crest-derived ganglia (..
  39. Fontana X, Hristova M, Da Costa C, Patodia S, Thei L, Makwana M, et al. c-Jun in Schwann cells promotes axonal regeneration and motoneuron survival via paracrine signaling. J Cell Biol. 2012;198:127-41 pubmed publisher
    ..These results reveal an unexpected function for c-Jun in SCs in response to axonal injury, and identify paracrine Ret signaling as an important mediator of c-Jun function in SCs during regeneration. ..
  40. Hamilton G, Gillingwater T. Spinal muscular atrophy: going beyond the motor neuron. Trends Mol Med. 2013;19:40-50 pubmed publisher
    ..Traditionally, reduced levels of SMN were thought to cause the selective death of lower motor neurons, leading to denervation and atrophy of skeletal muscles...
  41. Arber S. Motor circuits in action: specification, connectivity, and function. Neuron. 2012;74:975-89 pubmed publisher
    ..This Review highlights key examples of how developmental specification dictates organization of motor circuit connectivity and thereby controls movement. ..
  42. Subramanian N, Wetzel A, Dombert B, Yadav P, Havlicek S, Jablonka S, et al. Role of Na(v)1.9 in activity-dependent axon growth in motoneurons. Hum Mol Genet. 2012;21:3655-67 pubmed publisher
    ..Na(V)1.9 activation could therefore serve as a target for modulating axonal regeneration in motoneuron diseases such as SMA in which presynaptic activity of VGCCs is reduced. ..
  43. Chou H, Lai D, Huang C, McLennan I, Wang H, Wang P. BMP4 is a peripherally-derived factor for motor neurons and attenuates glutamate-induced excitotoxicity in vitro. PLoS ONE. 2013;8:e58441 pubmed publisher
    ..that Schwann cell- and muscle fiber-derived BMP4 proteins were anterogradely and retrogradely transported by motor neurons. Furthermore, BMP4 mRNA was down-regulated in nerves but up-regulated in skeletal muscles following nerve ..
  44. Song W, Song Y, Kincaid B, Bossy B, Bossy Wetzel E. Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1?. Neurobiol Dis. 2013;51:72-81 pubmed publisher
    ..Here, we report an improved technique to isolate, transfect, and culture rat spinal cord motor neurons. Using this improved system, we demonstrate that mutant SOD1(G93A) triggers a significant decrease in ..
  45. Roy A, Francius C, Rousso D, Seuntjens E, Debruyn J, Luxenhofer G, et al. Onecut transcription factors act upstream of Isl1 to regulate spinal motoneuron diversification. Development. 2012;139:3109-19 pubmed publisher
    ..Taken together, these data elucidate a comprehensive model wherein Onecut factors control multiple aspects of MN subtype diversification. They also shed light on the late roles of Isl1 in MN fate decision. ..
  46. Sareen D, Ebert A, Heins B, McGivern J, Ornelas L, Svendsen C. Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy. PLoS ONE. 2012;7:e39113 pubmed publisher
    ..using a virus-free plasmid-based approach-recapitulate the disease phenotype and generate significantly fewer motor neurons at later developmental time periods in culture compared to two separate control subject iPSC lines...
  47. Hanrieder J, Malmberg P, Lindberg O, Fletcher J, Ewing A. Time-of-flight secondary ion mass spectrometry based molecular histology of human spinal cord tissue and motor neurons. Anal Chem. 2013;85:8741-8 pubmed publisher
    ..imaging has been carried out at submicrometer resolution obtaining localization and characterization of spinal motor neurons based on their chemical fingerprint, including neurotransmitter precursors that serve as molecular indicators ..
  48. Wang W, Li L, Lin W, Dickson D, Petrucelli L, Zhang T, et al. The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons. Hum Mol Genet. 2013;22:4706-19 pubmed publisher
    ..evidence suggests that impaired mitochondrial dynamics likely contribute to the selective degeneration of motor neurons in SOD1-associated ALS...
  49. Shin J, Cho Y, Beirowski B, Milbrandt J, Cavalli V, DiAntonio A. Dual leucine zipper kinase is required for retrograde injury signaling and axonal regeneration. Neuron. 2012;74:1015-22 pubmed publisher
    ..These data demonstrate that DLK enhances regeneration by promoting a retrograde injury signal that is required for the activation of the neuronal proregenerative program. ..
  50. Tesla R, Wolf H, Xu P, Drawbridge J, Estill S, Huntington P, et al. Neuroprotective efficacy of aminopropyl carbazoles in a mouse model of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2012;109:17016-21 pubmed publisher
    ..Here, we provide evidence that an active analog of P7C3, known as P7C3A20, protects ventral horn spinal cord motor neurons from cell death in the G93A-SOD1 mutant mouse model of amyotrophic lateral sclerosis (ALS)...
  51. Amoroso M, Croft G, Williams D, O Keeffe S, Carrasco M, Davis A, et al. Accelerated high-yield generation of limb-innervating motor neurons from human stem cells. J Neurosci. 2013;33:574-86 pubmed publisher
    ..However, their widespread use even for intensely studied cell types like spinal motor neurons is hindered by the long duration and low yields of existing protocols for in vitro differentiation and by the ..
  52. Restani L, Giribaldi F, Manich M, Bercsényi K, Menendez G, Rossetto O, et al. Botulinum neurotoxins A and E undergo retrograde axonal transport in primary motor neurons. PLoS Pathog. 2012;8:e1003087 pubmed publisher
    ..tetanus and botulism have been ascribed to the different fate of the parental neurotoxins once internalised in motor neurons. Tetanus toxin (TeNT) is known to undergo transcytosis into inhibitory interneurons and block the release of ..
  53. Yamashita T, Hideyama T, Hachiga K, Teramoto S, Takano J, Iwata N, et al. A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology. Nat Commun. 2012;3:1307 pubmed publisher
    Both mislocalization of TDP-43 and downregulation of RNA-editing enzyme ADAR2 co-localize in the motor neurons of amyotrophic lateral sclerosis patients, but how they are linked is not clear...
  54. Fogarty M, Smallcombe K, Yanagawa Y, Obata K, Bellingham M, Noakes P. Genetic deficiency of GABA differentially regulates respiratory and non-respiratory motor neuron development. PLoS ONE. 2013;8:e56257 pubmed publisher
    ..when motor neuron numbers are being reduced, and when synaptic connections are being established onto and by motor neurons. In mice this occurs between embryonic (E) day 13 and birth (postnatal day 0)...
  55. Phatnani H, Guarnieri P, Friedman B, Carrasco M, Muratet M, O Keeffe S, et al. Intricate interplay between astrocytes and motor neurons in ALS. Proc Natl Acad Sci U S A. 2013;110:E756-65 pubmed publisher
    ALS results from the selective and progressive degeneration of motor neurons. Although the underlying disease mechanisms remain unknown, glial cells have been implicated in ALS disease progression...
  56. Bryson J, Hobbs C, Parsons M, Bosch K, Pandraud A, Walsh F, et al. Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Hum Mol Genet. 2012;21:3871-82 pubmed publisher
    In amyotrophic lateral sclerosis (ALS), the progressive loss of motor neurons is accompanied by extensive muscle denervation, resulting in paralysis and ultimately death...
  57. Liao B, Zhao W, Beers D, Henkel J, Appel S. Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS. Exp Neurol. 2012;237:147-52 pubmed publisher
    ..Thus, harnessing the neuroprotective potential of microglia may provide novel avenues for ALS therapies. ..
  58. Imlach W, Beck E, Choi B, Lotti F, Pellizzoni L, McCabe B. SMN is required for sensory-motor circuit function in Drosophila. Cell. 2012;151:427-39 pubmed publisher
    ..Unexpectedly, restoration of SMN in either muscles or motor neurons did not alter these phenotypes...
  59. Menelaou E, McLean D. A gradient in endogenous rhythmicity and oscillatory drive matches recruitment order in an axial motor pool. J Neurosci. 2012;32:10925-39 pubmed publisher
    ..Thus, within the zebrafish spinal cord, there are differences in the relative contribution of endogenous versus network-based rhythms to locomotion and these vary predictably according to order of recruitment. ..
  60. Witts E, Zagoraiou L, Miles G. Anatomy and function of cholinergic C bouton inputs to motor neurons. J Anat. 2014;224:52-60 pubmed publisher
    ..C boutons are large, cholinergic inputs to motor neurons which were first described over 40 years ago but whose source and function have until recently remained a ..
  61. Keller B, Volkening K, Droppelmann C, Ang L, Rademakers R, Strong M. Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism. Acta Neuropathol. 2012;124:733-47 pubmed publisher
    ..In this study, we evaluated the expression of 10 ALS-associated proteins in spinal motor neurons (MNs) in ALS patients with mutations in C9orf72 (C9orf72(GGGGCC)-ALS; n = 5), SOD1 (mtSOD1-ALS; n = 9), FUS/..
  62. Goodwin P, Sasaki J, Juo P. Cyclin-dependent kinase 5 regulates the polarized trafficking of neuropeptide-containing dense-core vesicles in Caenorhabditis elegans motor neurons. J Neurosci. 2012;32:8158-72 pubmed publisher
    ..distribution of neuropeptide-containing dense-core vesicles (DCVs) in Caenorhabditis elegans cholinergic motor neurons. In cdk-5 or cdka-1/p35 mutants, the predominantly axonal localization of DCVs containing INS-22 neuropeptides ..
  63. Liu H, Tjostheim S, DaSilva K, Taylor D, Zhao B, Rakhit R, et al. Targeting of monomer/misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis. J Neurosci. 2012;32:8791-9 pubmed publisher
    ..Our results support that SOD1 misfolding/aggregation plays a central role in SOD1-linked ALS pathogenesis and identifies monomeric/misfolded SOD1 as a therapeutic target for SOD1-related ALS. ..
  64. Jha R, Liu X, Chrenek R, Madsen J, Cardozo D. The postnatal human filum terminale is a source of autologous multipotent neurospheres capable of generating motor neurons. Neurosurgery. 2013;72:118-29; discussion 129 pubmed publisher
    ..Moreover, they have been induced to form motor neurons capable of innervating striated muscle in vitro...