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Neuroscience2019;41688-99.September 15, 2019Journal Article

Hyperexcitability in Cultured Cortical Neuron Networks from the G93A-SOD1 Amyotrophic Lateral Sclerosis Model Mouse and its Molecular Correlates.

Stefania Marcuzzo1, Benedetta Terragni2, Silvia Bonanno3, Davide Isaia4, Paola Cavalcante3, Cristina Cappelletti3, Emilio Ciusani5, Ambra Rizzo5, Giulia Regalia6, Natsue Yoshimura7, Giovanni Stefano Ugolini8, Marco Rasponi8, Giulia Bechi2, Massimo Mantegazza9, Renato Mantegazza3, Pia Bernasconi3, Ludovico Minati10
1Neurology IV -Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy. Electronic address: stefania.marcuzzo@istituto-besta.it.
2Neurophysiopathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy.
3Neurology IV -Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy.
4Neurology IV -Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy; Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Department of Development and Stem Cells, CNRS UMR7104, INSERM U964, Université de Strasbourg, 67404 Illkirch CU, Strasbourg, France.
5Laboratory of Clinical Pathology and Medical Genetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy.
6Neuroengineering and Medical Robotics Laboratory, Politecnico di Milano, Milan 20133, Italy; Currently working at Empatica srl, Milan 20144, Italy.
7World Research Hub Initiative (WRHI), Institute of Innovative Research, Tokyo Institute of Technology, Yokohama 226-8503, Japan.
8Department of Electronics, Information & Bioengineering, Politecnico di Milano, Milan 20133, Italy.
9Neurophysiopathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan 20133, Italy; Université Côte d'Azur, CNRS UMR7275, LabEx ICST, Institute of Molecular and Cellular Pharmacology, 06560 Valbonne-Sophia Antipolis, France.
10World Research Hub Initiative (WRHI), Institute of Innovative Research, Tokyo Institute of Technology, Yokohama 226-8503, Japan; Complex Systems Theory Department, Institute of Nuclear Physics, Polish Academy of Sciences (IFJ-PAN), 31-342 Kraków, Poland; Center for Mind/Brain Sciences (CIMeC), University of Trento, 38123 Trento, Italy.
PubMed DOI

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the corticospinal tract and leading to motor neuron death. According to a recent study, magnetic resonance imaging-visible changes suggestive of neurodegeneration seem absent in the motor cortex of G93A-SOD1 ALS mice. However, it has not yet been ascertained whether the cortical neural activity is intact, or alterations a…

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