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Séminaire impromptu - Enrico TongiorgiAn in vitro model to study neuronal atrophy and rescue in neurodevelopmental disorders: the case of Rett Syndrome

Abstract :


 Neuronal development is a complex multistep process that can be divided in several, partially overlapping stages from the initial establishment of polarization
, to specification of axon and growth of characteristic dendrite arbors, with a final maturation step including synaptogenesis and formation of dendritic spines. Numerous neuropsychiatric disorders are characterized by abnormal spine density and morphology, synapse loss, and aberrant dendrite morphology. We analyzed in detail the development of rat and mouse hippocampal neurons in vitro within the first 3 weeks, and defined 6 stages of development (with respect to the former 5 stages model by Dotti et al., 1988). We identified the subcellular localization, at specific time points and in response to stimuli, of components of mRNA trafficking and local translation machinery including ER and Golgi apparatuses. Thanks to this model in vitro we identified the changes in dendrite morphology, spines and synapses occurring in MeCP2 null-mice a model of Rett Syndrome, a neurodevelopmental disorder characterized by neuronal atrophy in absence of cell death and we tested drugs for the rescue of neuronal atrophy in Rett Syndrome.

 

Scientific focus :

Enrico Tongiorgi received his degree in Biology at the University of Pisa, Italy and a PhD in neurobiology at the ETH-Zurich, Switzerland. He was pre-doctoral fellow at the University of Heidelberg, Germany, short-term visiting scientist at MRC-Cambridge, UK and post-doc at the Intl. School of Advance Studies (SISSA)-Italy. He is now Associate Professor at the University of Trieste, Italy where he founded the Intl. Master Degree in Neuroscience and has been Director of the Brain Centre for Neuroscience.
Prof. Tongiorgi is Member of the Editorial Board of Scientific Reports and Frontiers in Molecular Neuroscience. He has been reviewer for grant agencies of UK, Poland, France, The Netherlands, Chile, the USA, Germany. He has been Member of the evaluation committee for AERES-France, Enterprise Ireland, and the Third-World Academy of Sciences (TWAS).

Prof. Tongiorgi’s work has been focused on dendritic mRNA trafficking and local translation of the neurotrophin Brain-derived neurotrophic factor (BDNF) and its role in neuronal development and neurological diseases with dendritic atrophy including depression, and epilepsy. More recently, he has developed an in vitro model the Rett Syndrome.