Selected Publications
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Stasimon Contributes to the Loss of Sensory Synapses and Motor Neuron Death in a Mouse Model of Spinal Muscular Atrophy.
Simon CM, Van Alstyne M, Lotti F, Bianchetti E, Tisdale S, Watterson DM, Mentis GZ, Pellizzoni L. Cell Reports (2019) 29:3885-3901. Pubmed |
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The Classical Complement Pathway Mediates Microglia-Dependent Remodeling of Spinal Motor Circuits during Development and in SMA.
Vukojicic A, Delestrée N, Fletcher EV, Pagiazitis JG, Sankaranarayanan S, Yednock TA, Barres BA, Mentis GZ. Cell Reports (2019) 29:3087-3100. Pubmed |
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Subtype Diversification and Synaptic Specificity of Stem Cell-Derived Spinal Interneurons.
Hoang PT, Chalif JI, Bikoff JB, Jessell TM, Mentis GZ, Wichterle H. Neuron (2018) 100:135-149. Pubmed |
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Dysregulation of Mdm2 and Mdm4 alternative splicing underlies motor neuron death in spinal muscular atrophy.
Van Alstyne M, Simon CM, Sardi SP, Shihabuddin LS, Mentis GZ, Pellizzoni L. Genes & Development (2018) 32:1045-1059. Pubmed |
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Preserving neuromuscular synapses in ALS by stimulating MuSK with a therapeutic agonist antibody.
Cantor S, Zhang W, Delestrée N, Remédio L, Mentis GZ, Burden SJ. Elife (2018) 7. pii: e34375. Pubmed |
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Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy.
Simon CM, Dai Y, Van Alstyne M, Koutsioumpa C, Pagiazitis JG, Chalif JI, Wang X, Rabinowitz JE, Henderson CE, Pellizzoni L, Mentis GZ. Cell Reports (2017) 21:3767-3780. Pubmed |
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Reduced sensory synaptic excitation impairs motor neuron function via Kv2.1 in spinal muscular atrophy.
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A stem cell model of the motor circuit uncouples motor neuron death from hyperexcitability induced by SMN deficiency.
Simon CM*, Janas AM*, Lotti F, Tapia JC, Pellizzoni L**, Mentis GZ**. * equal contribution, ** co-corresponding authors Cell Rep. (2016) 16:1416-1430. Pubmed |
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Sensory-derived glutamate regulates presynaptic inhibitory terminals in mouse spinal cord.
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Spinal inhibitory interneuron diversity delineates variant motor microcircuits.
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Diverging roles for Lrp4 and Wnt signaling in neuromuscular synapse development during evolution.
Remédio L, Gribble KD, Lee JK, Kim N, Hallock PT, Delestrée N, Mentis GZ, Froemke RC, Granato M, Burden SJ. Genes & Development (2016) 30:1058-69. Pubmed |
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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.
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Activity regulates the incidence of heteronymous sensory-motor connections.
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SMN is essential for the biogenesis of U7 small nuclear ribonucleoprotein and 3'-end formation of histone mRNAs.
Tisdale S, Lotti F, Saieva L, Van Meerbeke JP, Crawford TO, Sumner CJ, Mentis GZ, Pellizzoni L. Cell Rep. (2013) 5:1187-1195. Pubmed |
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An SMN-dependent U12 splicing event essential for motor circuit function.
Lotti F, Imlach WL, Saieva L, Beck ES, Hao le T, Li DK, Jiao W, Mentis GZ, Beattie CE, McCabe BD, Pellizzoni L. Cell (2012) 151:440-454. Pubmed |
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Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy.
Mentis GZ, Blivis D, Liu W, Drobac E, Crowder ME, Kong L, Alvarez FJ, Sumner CJ, O'Donovan MJ. Neuron (2011) 69:453-467. Pubmed |
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Mechanisms regulating the specificity and strength of muscle afferent inputs in the spinal cord.
Mentis GZ, Alvarez FJ, Shneider NA, Siembab VC, O'Donovan MJ. Ann N Y Acad Sci. (2010) 1198:220-230. Pubmed |