DFG founded research groups » Research Group Simon » Publications

Research Group Simon


  • Kong L., Hassinan, C., Gerstner, F., Buettner, J.M., Petigrow, J., Valdivia, D., Chan-Cortés, M., Mistri, A., Cao, A., McGaugh, S.A., Denton, M., Brown, S., Ross, J., Schwab, M., Simon, C.M., Sumner, C. (2023).
    Boosting neuregulin 1 type-III expression hastens SMA motor axon maturation
    Acta neuropathol commun (accepted)
  • Hennlein, L., Ghanawi, H., Gerstner, F., Palominos Garcia, E., Yildirim, E., Saal-Bauernschubert, L., Moradi, M., Deng, C., Klein, T., Appenzeller, S., Sauer, M., Briese, M., Simon, C.M., Sendtner, M., Jablonka, S. (2023).
    Plastin 3 rescues cell surface translocation and activation of TrkB in spinal muscular atrophy.
    J Cell Biol 222. 10.1083/jcb.202204113.
  • Buettner, J.M., Sowoidnich, L., Gerstner, F., Blanco-Redondo, B., Hallermann, S., Simon, C.M. (2022).
    p53-dependent c-Fos expression is a marker but not executor for motor neuron death in spinal muscular atrophy mouse models.
    Front. Cell. Neurosci., doi.org/10.3389/fncel.2022.103
  • Tisdale, S., Van Alstyne, M., Simon, C.M., Mentis, G.Z., and Pellizzoni, L. (2022).
    SMN controls neuromuscular junction integrity through U7 snRNP.
    Cell Rep 40, 111393. 10.1016/j.celrep.2022.111393.
  • Buettner, J.M., Kirmann, T., Mentis, G.Z., Hallermann, S., and Simon, C.M. (2022).
    Laser microscopy acquisition and analysis of premotor synapses in the murine spinal cord.
    STAR Protoc 3, 101236. 10.1016/j.xpro.2022.101236.
  • Buettner, J.M., Sime Longang, J.K., Gerstner, F., Apel, K.S., Blanco-Redondo, B., Sowoidnich, L., Janzen, E., Langenhan, T., Wirth, B., and Simon, C.M. (2021).
    Central synaptopathy is the most conserved feature of motor circuit pathology across spinal muscular atrophy mouse models.
    iScience 24, 103376. 10.1016/j.isci.2021.103376.
  • Kong, L., Valdivia, D.O., Simon, C.M., Hassinan, C., Delestree, N., Ramos, D., Park J.H., Celeste, P., Xu X., Crowder, M., Gyrzb, C., King, Z., Petrillo, M., Swoboda, K., Davis, C., Lutz, C., Weetall, M., Naryshkin, N., Crawford, T.O., Mentis, G.Z., Sumner, C.J. (2021).
    Impaired prenatal motor axon development necessitates early therapeutic intervention in severe SMA.
    Sci. Transl. Med. 13, eabb6871
  • Simon, C.M*., Blanco-Redondo, B., Buettner, J.M., Pagiazitis, J.G., Fletcher, E.V., Sime Longang, J.K., and Mentis, G.Z. (2021).
    Chronic Pharmacological Increase of Neuronal Activity Improves Sensory-Motor Dysfunction in Spinal Muscular Atrophy Mice.
    J Neurosci 41, 376-389, *Correspondence
  • Simon, C.M., Van Alstyne, M., Lotti, F., Bianchetti, E., Tisdale, S., Watterson, D.M., Mentis, G.Z., and Pellizzoni, L. (2019).
    Stasimon Contributes to the Loss of Sensory Synapses and Motor Neuron Death in a Mouse Model of Spinal Muscular Atrophy.
    Cell Rep 29, 3885-3901 e3885.
  • Van Alstyne M, Simon CM, Sardi SP, Shihabuddin LS, Mentis GZ, Pellizzoni L. (2018)
    Dysregulation of Mdm2 and Mdm4 alternative splicing underlies motor neuron death in spinal muscular atrophy.
    Genes Dev. Aug 1;32(15-16):1045-1059
  • Simon CM, Dai Y, Van Alstyne M, Koutsioumpa C, Pagiazitis JG, Chalif JI, Wang X, Rabinowitz JE, Pellizzoni L, Henderson CE, Mentis GZ (2017)
    Converging mechanisms of p53 activation drive motor neuron degeneration in spinal muscular atrophy.
    Cell Reports 21, 3767–3780
  • Fletcher EV, Simon, CM, Pagiazitis, JG, Chalif, JI, Vukojicic, A, Drobac, E, Wang, X, and Mentis, GZ (2017)
    Reduced sensory synaptic excitation impairs motor neuron function via Kv2.1 in spinal muscular atrophy.
    Nature neuroscience 20, 905-916
  • Simon CM, Janas A.M, Lotti F, Tabia JC, Pellizzoni L, Mentis GZ (2016)
    A stem cell model of the motor circuit uncouples motor neuron death from hyperexcitability induced by SMN deficiency
    Cell Reports 16, 1416–1430
  • Jesse CM, Bushuven E, Tripathi P, Chandrasekar A, Simon CM, Drepper C, Yamoah A, Dreser A, Katona I, Johann S, Beyer C, Wagner S, Grond M, Nikolin S, Anink J, Troost D, Sendtner M, Goswami A, Weis J (2016)
    ALS-associated endoplasmic reticulum proteins in denervated skeletal muscle: Implications for motor neuron disease pathology.
    Brain Pathology doi: 10.1111/bpa.12453
  • Mendelsohn AI, Simon CM, Abbott LF, Mentis GZ, Jessell T (2015
    Activity Regulates the Incidence of Heteronymous Sensory-Motor Connections.
    Neuron Jul 1;87(1):111-23
  • Simon CM, Gunnersen JM, Rauskolb S, Holtmann B, Drepper C, Braga M, Wiese S, Jablonka S, Puehringer D, Dombert B, Zielasek J, Hoeflich A, Silani V, Wolf E, Kneitz S, Sommer C, Toyka K, Sendtner M (2015)
    Dysregulated IGFBP5 expression causes axon degeneration and motoneuron loss in diabetic neuropathy.
    Acta Neuropathol. 10.1007/s00401-015-1446-8
  • de Nooij JC, Simon CM, Simon A, Doobar S, Steel KP, Banks RW, Mentis GZ, Bewick GS, Jessell T (2015)
    The PDZ-domain protein Whirlin facilitates mechanosensory signaling in mammalian proprioceptors.
    J Neurosci. 35(7):3073-84
  • Dombert B, Sivadasan R, Simon CM, Jablonka S, Sendtner M (2014)
    Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons.
    PLoS One, 10; 9(10):e110846
  • Simon CM, Jablonka, S, Ruiz, R, Tabares, L, Sendtner, M (2010)
    Ciliary neurotrophic factor-induced sprouting preserves motor function in a mouse model of spinal muscular atrophy.
    Hum. Mol. Genet., 19 973-986
  • Fischer, M, Pereira, PM, Holtmann, B, Simon, CM, Hanauer, A, Heisenberg, M, Sendtner, M (2009)
    P90 ribosomal s6 kinase 2 negatively regulates axon growth in motoneurons.
    Mol. Cell Neurosci., 42 134-141