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1. A cell-penetrant peptide blocking C9ORF72 -repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins

Author

Lydia M. Castelli, Ya-Hui Lin, Alvaro Sanchez-Martinez, Aytaç Gül, Kamallia Mohd Imran, Adrian Higginbottom, Santosh Kumar Upadhyay, Nóra M. Márkus, Raquel Rua Martins, Johnathan Cooper-Knock, Claire Montmasson, Rebecca Cohen, Amy Walton, Claudia S. Bauer, Kurt J. De Vos, Richard J. Mead, Mimoun Azzouz, Cyril Dominguez, Laura Ferraiuolo, Pamela J. Shaw, Alexander J. Whitworth, and Guillaume M. Hautbergue

Subjects
General Medicine
Abstract

Hexanucleotide repeat expansions in C9ORF72 are the most common genetic cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Studies have shown that the hexanucleotide expansions cause the noncanonical translation of C9ORF72 transcripts into neurotoxic dipeptide repeat proteins (DPRs) that contribute to neurodegeneration. We show that a cell-penetrant peptide blocked the nuclear export of C9ORF72 -repeat transcripts in HEK293T cells by competing with the interaction between SR-rich splicing factor 1 (SRSF1) and nuclear export factor 1 (NXF1). The cell-penetrant peptide also blocked the translation of toxic DPRs in neurons differentiated from induced neural progenitor cells (iNPCs), which were derived from individuals carrying C9ORF72 -linked ALS mutations. This peptide also increased survival of iNPC-differentiated C9ORF72-ALS motor neurons cocultured with astrocytes. Oral administration of the cell-penetrant peptide reduced DPR translation and rescued locomotor deficits in a Drosophila model of mutant C9ORF72-mediated ALS/FTD. Intrathecal injection of this peptide into the brains of ALS/FTD mice carrying a C9ORF72 mutation resulted in reduced expression of DPRs in mouse brains. These findings demonstrate that disrupting the production of DPRs in cellular and animal models of ALS/FTD might be a strategy to ameliorate neurodegeneration in these diseases.

Published
2023
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2. Convergence of adenosine and GABA signaling for synapse stabilization during development

Author

Carla G. Silva, Marianne Renner, Jessica C. Pressey, Sabine Lévi, Sofia Alçada-Morais, Shiva K. Tyagarajan, Francisco Q. Gonçalves, Monique Esclapez, Christophe Leterrier, Ferran Gomez-Castro, Xavier Nicol, Nicolas Gervasi, Marion Russeau, Ricardo Gouveia Rodrigues, Stefania Zappettini, Rodrigo A. Cunha, Marta Figueiredo, Christophe Bernard, Olivier Thoumine, Eszter Szabó, Claire Montmasson, Ghislaine Caillol, Angelo R. Tomé, Paula M. Canas, Rafael Luján, Paula Agostinho, University of Zurich, Bernard, Christophe, Lévi, Sabine, Institut du Fer à Moulin (IFM - Inserm U1270 - SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Institut de Neurosciences des Systèmes (INS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), University of Toronto, University of Coimbra [Portugal] (UC), Centre interdisciplinaire de recherche en biologie (CIRB), Labex MemoLife, École normale supérieure - Paris (ENS Paris), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Collège de France (CdF (institution))-Ecole Superieure de Physique et de Chimie Industrielles de la Ville de Paris (ESPCI Paris), Université Paris sciences et lettres (PSL)-École normale supérieure - Paris (ENS Paris), Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), University hospital of Zurich [Zurich], Institut de neurophysiopathologie (INP), Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Interdisciplinary Institute for Neuroscience [Bordeaux] (IINS), Université de Bordeaux (UB)-Centre National de la Recherche Scientifique (CNRS), Institut de la Vision, Centre National de la Recherche Scientifique (CNRS)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Universidad de Castilla-La Mancha (UCLM), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), École normale supérieure - Paris (ENS-PSL), Université Paris sciences et lettres (PSL)-École normale supérieure - Paris (ENS-PSL), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), and Universidad de Castilla-La Mancha = University of Castilla-La Mancha (UCLM)

Subjects
Male, Adenosine, Receptor, Adenosine A2A, [SDV]Life Sciences [q-bio], Adenosine A2A receptor, 10050 Institute of Pharmacology and Toxicology, Nerve Tissue Proteins, 610 Medicine & health, Hippocampus, Synapse, 03 medical and health sciences, chemistry.chemical_compound, Mice, 0302 clinical medicine, Adenosine Triphosphate, Cognition, Postsynaptic potential, medicine, Cyclic AMP, Animals, Phosphorylation, gamma-Aminobutyric Acid, 030304 developmental biology, Neurons, 0303 health sciences, 1000 Multidisciplinary, Multidisciplinary, Gephyrin, biology, Chemistry, Membrane Proteins, Receptors, GABA-A, Cyclic AMP-Dependent Protein Kinases, Cell biology, Adenosine A2 Receptor Antagonists, Synapses, biology.protein, GABAergic, 570 Life sciences, Calcium, Signal transduction, Adenosine triphosphate, 030217 neurology & neurosurgery, medicine.drug, Signal Transduction
Abstract

Synapse stabilization Early in brain development, neurons connect to each other enthusiastically. With development, an overabundance of synapses is winnowed down to refine efficiently connected circuits. Inactive synapses are prime targets for elimination, whereas active synapses tend to be retained. Gomez-Castro et al . took a closer look at how those choices are made (see the Perspective by Blum and Lopes). When postsynaptic adenosine receptors are muted or do not find enough extracellular adenosine, synapses get eliminated. Neurotransmitter-dependent signaling pathways drive protein kinase A to phosphorylate the postsynaptic scaffolding molecule gephyrin. Together with a partner synaptogenic membrane protein, gephyrin is required for the stabilization of γ-aminobutyric acid receptors. Adenosine receptors thus detect synaptic activity and in turn drive the stabilization of synapses that produce such activity. —PJH

Published
2021
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