Neurodevelopmental disorders
Translational program
Research
We study neurodevelopmental disorders with the ultimate aim to identify key molecular and cellular mechanisms involved in establishing neuronal circuits underlying social cognition and behavior. We currently focus on the study of transcription factors whose mutations lead to speech and language disorder and to microcephaly and intellectual disability.
We aim to dissect the molecular targets of these transcription factors and their roles in neuronal physiology and behavior. In order to tackle these issues, we employ state of the art complementary methods in molecular genetics, cell biology, electrophysiology and optogenetics in genetic mouse models and human iPSC-derived neurons. Eventually our research should contribute to a better understanding of complex neurodevelopmental syndromes which affect the ‘social brain’ such as autisms or related disorders.
People
- Matthias Groszer, CRCN Inserm, Principal investigator
- Hudson Bezerra, Postdoctoral scientist
Long-term development of human iPSC-derived pyramidal neurons quantified after transplantation into the neonatal mouse cortex.
D’Alessio R, Koukouli F, Blanchard S, Catteau J, Raïs C, Lemonnier T, Féraud O, Bennaceur-Griscelli A, Groszer M, Maskos U.
Dev Biol. 2020 May 1;461(1):86-95.
PMID:31982375 
TBR2 coordinates neurogenesis expansion and precise microcircuit organization via Protocadherin 19 in the mammalian cortex.
Lv X, Ren SQ, Zhang XJ, Shen Z, Ghosh T, Xianyu A, Gao P, Li Z, Lin S, Yu Y, Zhang Q, Groszer M, Shi SH.
Nat Commun. 2019 Sep 2;10(1):3946.
PMID:31477701
ATP6AP2 variant impairs CNS development and neuronal survival to cause fulminant neurodegeneration.
Hirose T, Cabrera-Socorro A, Chitayat D, Lemonnier T, Féraud O, Cifuentes-Diaz C, Gervasi N, Mombereau C, Ghosh T, Stoica L, Bacha JDA, Yamada H, Lauterbach MA, Guillon M, Kaneko K, Norris JW, Siriwardena K, Blasér S, Teillon J, Mendoza-Londono R, Russeau M, Hadoux J, Ito S, Corvol P, Matheus MG, Holden KR, Takei K, Emiliani V, Bennaceur-Griscelli A, Schwartz CE, Nguyen G, Groszer M.
J Clin Invest. 2019 Apr 15;129(5):2145-2162.
PMID:30985297
AUTS2 isoforms control neuronal differentiation.
Monderer-Rothkoff G, Tal N, Risman M, Shani O, Nissim-Rafinia M, Malki-Feldman L, Medvedeva V, Groszer M, Meshorer E, Shifman S.
Mol Psychiatry. 2019 Apr 5.
PMID:30953002
Mice carrying a humanized Foxp2 knock-in allele show region-specific shifts of striatal Foxp2 expression levels.
Schreiweis C, Irinopoulou T, Vieth B, Laddada L, Oury F, Burguière E, Enard W, Groszer M.
Cortex. 2019 Sep;118:212-222.
PMID:30808549
Altered social behavior in mice carrying a cortical Foxp2 deletion.
Medvedeva VP, Rieger MA, Vieth B, Mombereau C, Ziegenhain C, Ghosh T, Cressant A, Enard W, Granon S, Dougherty JD, Groszer M.
Hum Mol Genet. 2019 Mar 1;28(5):701-717.
PMID:30357341
Thalamic WNT3 Secretion Spatiotemporally Regulates the Neocortical Ribosome Signature and mRNA Translation to Specify Neocortical Cell Subtypes.
Matthew L Kraushar, Barbara Viljetic, H R Sagara Wijeratne, Kevin Thompson, Xinfu Jiao, Jack W Pike, Vera Medvedeva, Matthias Groszer, Megerditch Kiledjian, Ronald P Hart, Mladen-Roko Rasin
J Neurosci. 2015 Aug 5;35(31):10911-26.
PMID:26245956
Humanized Foxp2 accelerates learning by enhancing transitions from declarative to procedural performance.
Christiane Schreiweis, Ulrich Bornschein, Eric Burguière, Cemil Kerimoglu, Sven Schreiter, Michael Dannemann, Shubhi Goyal, Ellis Rea, Catherine A French, Rathi Puliyadi, Matthias Groszer, Simon E Fisher, Roger Mundry, Christine Winter, Wulf Hevers, Svante Pääbo, Wolfgang Enard, Ann M Graybiel
Proc Natl Acad Sci U S A. 2014 Sep 30;111(39):14253-8.
PMID:25225386
MicroRNAs establish robustness and adaptability of a critical gene network to regulate progenitor fate decisions during cortical neurogenesis.
Tanay Ghosh, Julieta Aprea, Jeannette Nardelli, Hannes Engel, Christian Selinger, Cedric Mombereau, Thomas Lemonnier, Imane Moutkine, Leslie Schwendimann, Martina Dori, Theano Irinopoulou, Alexandra Henrion-Caude, Arndt G Benecke, Sebastian J Arnold, Pierre Gressens, Federico Calegari, Matthias Groszer
Cell Rep. 2014 Jun 26;7(6):1779-88.
PMID:24931612
Transcriptome sequencing during mouse brain development identifies long non-coding RNAs functionally involved in neurogenic commitment.
Julieta Aprea, Silvia Prenninger, Martina Dori, Tanay Ghosh, Laura Sebastian Monasor, Elke Wessendorf, Sara Zocher, Simone Massalini, Dimitra Alexopoulou, Mathias Lesche, Andreas Dahl, Matthias Groszer, Michael Hiller, Federico Calegari
EMBO J. 2013 Dec 11;32(24):3145-60.
PMID:24240175
