Experts and Doctors on motor neurons in Boston, Massachusetts, United States


Locale: Boston, Massachusetts, United States
Topic: motor neurons

Top Publications

  1. Bergeron L, Perez G, Macdonald G, Shi L, Sun Y, Jurisicova A, et al. Defects in regulation of apoptosis in caspase-2-deficient mice. Genes Dev. 1998;12:1304-14 pubmed
    ..Thus, caspase-2 acts both as a positive and negative cell death effector, depending upon cell lineage and stage of development. ..
  2. 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
    ..We propose a model in which CDK-5 regulates DCV polarity by both promoting DCV trafficking in axons and preventing dynein-dependent DCV trafficking into dendrites. ..
  3. Silva D, Dikkes P, Barnes M, Lopez M. Decreased motoneuron survival in Igf2 null mice after sciatic nerve transection. Neuroreport. 2009;20:1414-8 pubmed publisher
    ..Neuronal survival was significantly improved in Igf2 mice when IGF2 was administered. These results support the role of IGF2 in neonatal motor neuron survival. ..
  4. Kasselman L, Shefner J, Rutkove S. Motor unit number estimation in the rat tail using a modified multipoint stimulation technique. Muscle Nerve. 2009;40:115-21 pubmed publisher
  5. Wang Z, Li L, Goulding M, Frank E. Early postnatal development of reciprocal Ia inhibition in the murine spinal cord. J Neurophysiol. 2008;100:185-96 pubmed publisher
    ..This suggests either that Pax6 is not required for the phenotypic development of all V1-derived spinal interneurons or that these inhibitory interneurons are not derived from V1 precursors. ..
  6. Sieburth D, Madison J, Kaplan J. PKC-1 regulates secretion of neuropeptides. Nat Neurosci. 2007;10:49-57 pubmed
    ..Similar neuropeptide secretion defects were found in mutants lacking unc-31 (encoding the protein CAPS) or unc-13 (encoding Munc13). These results suggest that PKC-1 selectively regulates DCV release from neurons...
  7. Goodwin P, Juo P. The scaffolding protein SYD-2/Liprin-? regulates the mobility and polarized distribution of dense-core vesicles in C. elegans motor neurons. PLoS ONE. 2013;8:e54763 pubmed publisher
    ..This study shows that SYD-2 promotes bi-directional mobility of DCVs and identifies SYD-2 as a novel regulator of DCV trafficking and polarized distribution. ..
  8. Molyneaux B, Arlotta P, Hirata T, Hibi M, Macklis J. Fezl is required for the birth and specification of corticospinal motor neurons. Neuron. 2005;47:817-31 pubmed
  9. Benson T, Lee D, Brown M. Tensor tympani motoneurons receive mostly excitatory synaptic inputs. Anat Rec (Hoboken). 2013;296:133-45 pubmed publisher
    ..Having a high proportion of excitatory inputs, the TTMN innervation is like that of stapedius motoneurons but proportionately different from other types of motoneurons...

More Information


  1. Ozdinler P, Macklis J. IGF-I specifically enhances axon outgrowth of corticospinal motor neurons. Nat Neurosci. 2006;9:1371-81 pubmed
    ..These experiments define specific controls over directed differentiation of CSMN, indicate a distinct role of IGF-I in CSMN axon outgrowth during development, and might enable control over CSMN derived from neural precursors. ..
  2. Westgaard R, De Luca C. Motor control of low-threshold motor units in the human trapezius muscle. J Neurophysiol. 2001;85:1777-81 pubmed
    ..We speculate that Renshaw cell inhibition contributes to the observed deviation of the low-threshold motor units from the hierarchical onion-skin pattern...
  3. Faust J, Rodman J, Daniel P, Dice J, Bronson R. Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis. J Biol Chem. 1994;269:10150-5 pubmed
    ..The accumulation of the vacuolar subunit c in this model may prove to be a marker for distinguishing different forms of NCLs. ..
  4. Ko J, Humbert S, Bronson R, Takahashi S, Kulkarni A, Li E, et al. p35 and p39 are essential for cyclin-dependent kinase 5 function during neurodevelopment. J Neurosci. 2001;21:6758-71 pubmed
    ..Our data strongly suggest that p35 and p39 are essential for Cdk5 activity during the development of the nervous system. Thus, p35 and p39 are likely to be the principal, if not the only, activators of Cdk5. ..
  5. Mukerji S, Brown M, Lee D. A morphologic study of Fluorogold labeled tensor tympani motoneurons in mice. Brain Res. 2009;1278:59-65 pubmed publisher
    ..The mean size of mouse TTMNs was 13.0 microm (minor axis) and 23.5 microm (major axis). Compared with studies of TTMNs in larger species (cats and rats), mouse TTMNs are both fewer in number and smaller in size...
  6. Wills Z, Bateman J, Korey C, Comer A, Van Vactor D. The tyrosine kinase Abl and its substrate enabled collaborate with the receptor phosphatase Dlar to control motor axon guidance. Neuron. 1999;22:301-12 pubmed
    ..These data suggest that Dlar, Abl, and Ena define a phosphorylation state-dependent switch that controls growth cone behavior by transmitting signals at the cell surface to the actin cytoskeleton. ..
  7. Lee J, Kannagi M, Ferrante R, Kowall N, Ryu H. Activation of Ets-2 by oxidative stress induces Bcl-xL expression and accounts for glial survival in amyotrophic lateral sclerosis. FASEB J. 2009;23:1739-49 pubmed publisher
    ..This survival pathway may contribute to the glial survival and activation seen in the spinal cord of ALS patients. ..
  8. Michalak S, Whitman M, Park J, Tischfield M, Nguyen E, Engle E. Ocular Motor Nerve Development in the Presence and Absence of Extraocular Muscle. Invest Ophthalmol Vis Sci. 2017;58:2388-2396 pubmed publisher
    ..These data suggest that intermediate targets in the mesenchyme provide cues necessary for appropriate targeting of ocular motor axons to the orbit, while EOM cues are responsible for terminal branching and motor neuron survival. ..
  9. Miller N, Feng Z, Edens B, Yang B, Shi H, Sze C, et al. Non-aggregating tau phosphorylation by cyclin-dependent kinase 5 contributes to motor neuron degeneration in spinal muscular atrophy. J Neurosci. 2015;35:6038-50 pubmed publisher
    ..Altogether, our findings suggest a novel mechanism for SMA pathogenesis in which hyperphosphorylation of non-aggregating tau by Cdk5 contributes to motor neuron degeneration. ..
  10. Higashi Kovtun M, Mosca T, Dickman D, Meinertzhagen I, Schwarz T. Importin-beta11 regulates synaptic phosphorylated mothers against decapentaplegic, and thereby influences synaptic development and function at the Drosophila neuromuscular junction. J Neurosci. 2010;30:5253-68 pubmed publisher
    ..Thus, importin-beta11 function interacts with the BMP pathway to regulate a pool of pMAD that must be present at the presynapse for its proper development and function. ..
  11. Thor S, Thomas J. Motor neuron specification in worms, flies and mice: conserved and 'lost' mechanisms. Curr Opin Genet Dev. 2002;12:558-64 pubmed
    ..By comparing findings from Caenorhabditis elegans, Drosophila and vertebrate model systems, it is apparent that both evolutionarily conserved and non-conserved mechanisms are used. ..
  12. Bateman J, Shu H, Van Vactor D. The guanine nucleotide exchange factor trio mediates axonal development in the Drosophila embryo. Neuron. 2000;26:93-106 pubmed
    ..Moreover, reduction of trio activity potentiates the phenotype of mutations in the LAR homolog Dlar, suggesting that these proteins collaborate in orchestrating the cytoskeletal events that underlie normal axonogenesis. ..
  13. Ozdinler P, Benn S, Yamamoto T, Güzel M, Brown R, Macklis J. Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G?³A transgenic ALS mice. J Neurosci. 2011;31:4166-77 pubmed publisher
    ..These results provide a foundation for detailed investigation of CSMN/SCPN vulnerability and toward potential CSMN therapeutics in ALS. ..
  14. Muroyama Y, Fujiwara Y, Orkin S, Rowitch D. Specification of astrocytes by bHLH protein SCL in a restricted region of the neural tube. Nature. 2005;438:360-3 pubmed
    ..They further indicate that acquisition of embryonic glial subtype identity might be regulated by genetic interactions between SCL and the transcription factor Olig2 in the ventral neural tube. ..
  15. Krivickas L, Hochberg F, Freeman S. Chronic inflammatory demyelinating polyradiculoneuropathy with tumefactive central demyelination. Muscle Nerve. 2006;33:283-8 pubmed
    ..The mass and hand weakness improved following steroid therapy. The combination of CIDP and central demyelination with mass effect broadens the spectrum of demyelinating disease in association with CIDP...
  16. Sieburth D, Ch ng Q, Dybbs M, Tavazoie M, Kennedy S, Wang D, et al. Systematic analysis of genes required for synapse structure and function. Nature. 2005;436:510-7 pubmed
    ..Twenty-four genes encoded proteins that were localized to presynaptic specializations. Loss-of-function mutations in 12 genes caused defects in presynaptic structure...
  17. Wills Z, Marr L, Zinn K, Goodman C, Van Vactor D. Profilin and the Abl tyrosine kinase are required for motor axon outgrowth in the Drosophila embryo. Neuron. 1999;22:291-9 pubmed
    ..Moreover, the phenotype of a double mutant suggests that these components function together to control axonal outgrowth. ..
  18. Pack Chung E, Kurshan P, Dickman D, Schwarz T. A Drosophila kinesin required for synaptic bouton formation and synaptic vesicle transport. Nat Neurosci. 2007;10:980-9 pubmed
    ..Our data thus indicate that Imac transports components required for synaptic maturation and provide insight into presynaptic maturation as a process that can be differentiated from axon outgrowth and targeting. ..