Review
Biochemistry & Molecular Biology
Amir Dudai, Nadav Yayon, Hermona Soreq, Michael London
Summary: The urgent task of neuroscience is to understand how neuronal circuits operate, what causes them to fail, and how they can be repaired. This review focuses on a specific type of cortical neurons marked by the expression of VIP and ChAT, which can release both GABA and ACh. Despite their sparse population, these neurons may have a significant impact on cortical circuit function.
JOURNAL OF NEUROCHEMISTRY
(2021)
Article
Neurosciences
Gabrielle Devienne, Sandrine Picaud, Ivan Cohen, Juliette Piquet, Ludovic Tricoire, Damien Testa, Ariel A. Di Nardo, Jean Rossier, Bruno Cauli, Bertrand Lambolez
Summary: Inhibition of parvalbumin-expressing (PV) interneurons in adult mice can induce perineuronal net (PNN) regression, while activation of these neurons does not reduce PNN density. The density of PNN in the adult cortex is regulated by local changes in network activity, which can be triggered through modulation of PV interneurons.
JOURNAL OF NEUROSCIENCE
(2021)
Article
Neurosciences
Wenxi Xing, Ana Dolabela de Lima, Thomas Voigt
Summary: Research shows that the number of GABAergic interneurons affects the proliferation of glutamatergic projection neurons and remains constant throughout the developmental process of cultured networks. Additionally, the number of GABA neurons may also impact the excitatory/inhibitory balance of network burst activity and the development of spontaneous activity.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
Article
Genetics & Heredity
Sathish Venkataramanappa, Dagmar Schuetz, Friederike Saaber, Praveen Ashok Kumar, Philipp Abe, Stefan Schulz, Ralf Stumm
Summary: Mutations in the DONSON gene have been linked to skeletal abnormalities and microcephaly. Deletion of Donson in cortical progenitors led to extensive cell death and abnormalities in cortical development. Targeting Donson may be a potential strategy for experimental and therapeutic purposes.
Editorial Material
Clinical Neurology
Thiviya Selvanathan, Mireille Guillot, Helen M. Branson, Vann Chau, Edmond N. Kelly, Steven P. Miller
Summary: Malformations of cortical development (MCD) are a rare group of disorders caused by genetic, metabolic, infectious, or vascular factors that disrupt the development of the cerebral cortex. They are typically detected with brain MRI when symptomatic, but recent advances allow for detection during fetal or neonatal periods. This study presents neuroimaging findings and neurodevelopmental outcomes of a very preterm infant with incidentally detected MCD on neonatal research brain MRI.
Article
Multidisciplinary Sciences
Christina M. Cerkevich, Jean-Alban Rathelot, Peter L. Strick
Summary: This study examines the cortical control of a laryngeal muscle, essential for vocalization in marmosets and macaques. The researchers find that the expansion of descending output from the premotor areas, particularly ventral area 6 and supplementary motor area, contributes to the enhanced vocal motor abilities in marmosets.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Review
Clinical Neurology
Nancy R. Mack, Suixin Deng, Sha-Sha Yang, Yousheng Shu, Wen-Jun Gao
Summary: This article reviews studies on the relationship between changes in neural activity in the prefrontal cortex and avoidance behavior, as well as recent research on the unique contribution of specific prefrontal cortex circuits and cell types to anxiety-related avoidance behaviors. The integration of different pathways, particularly interaction between excitatory and inhibitory neurons, provides an exciting opportunity for understanding anxiety.
Article
Neurosciences
Sandra U. Okoro, Roman U. Goz, Brigdet W. Njeri, Madhumita Harish, Catherine F. Ruff, Sarah E. Ross, Charles Gerfen, Bryan M. Hooks
Summary: This research demonstrates the relationship between synaptic connections and activation patterns of neurons in the motor cortex (M1) of the brain. Inputs from sensory cortex and thalamus target different types of M1 interneurons in a specific manner. In the regulation of M1 output, different types of neurons are activated in a specific way and circuit.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Medicine, Research & Experimental
Weihua Ding, Lukas Fischer, Qian Chen, Ziyi Li, Liuyue Yang, Zerong You, Kun Hu, Xinbo Wu, Xue Zhou, Wei Chao, Peter Hu, Tewodros Mulugeta Dagnew, Daniel M. Dubreuil, Shiyu Wang, Suyun Xia, Caroline Bao, Shengmei Zhu, Lucy Chen, Changning Wang, Brian Wainger, Peng Jin, Jianren Mao, Guoping Feng, Mark T. Harnett, Shiqian Shen
Summary: Cortical neural dynamics mediate information processing for the cerebral cortex, playing a crucial role in various biological processes and diseases. Spontaneous pain in neuropathic pain activates a specific area within the primary somatosensory cortex, resulting in synchronized neural dynamics. Manipulating the cortical network or applying clinically effective pain therapies can attenuate this synchronization and alleviate pain-like behavior.
JOURNAL OF CLINICAL INVESTIGATION
(2023)
Article
Biology
Sabrina A. Jones, Jacob H. Barfield, V. Kindler Norman, Woodrow L. Shew
Summary: Naturally occurring body movements and collective neural activity exhibit scale-free dynamics, with fractal spatiotemporal structure. Scale-free brain activity and scale-free behavior have been studied separately, without a unified explanation. We show that mouse behavior and neurons in the visual cortex exhibit strongly related scale-free dynamics, with stochastic competition among neural subsets. Our computational model incorporating cell-type-specific circuit structure provides a new explanation for these findings, establishing the neural underpinnings of scale-free behavior and the relevance of scale-free neural activity to behavior.
Review
Neurosciences
Iris A. Speigel, Hugh C. Hemmings Jr.
Summary: General anesthetics alter synaptic patterns of excitation and inhibition, disrupting brain processes involved in consciousness. Inhibitory interneurons play a crucial role in regulating specific neuronal networks, but the impact of anesthetics on these interneuron subtypes is still unknown. This review explores the synaptic architecture of cortical and interneuron subtypes, their contributions to different forms of inhibition, and the molecular and cellular targets in these circuits that are sensitive to anesthetics.
FRONTIERS IN SYNAPTIC NEUROSCIENCE
(2022)
Article
Cell Biology
Ming-Shu Xu, Lei-Miao Yin, Ai-Fang Cheng, Ying-Jie Zhang, Di Zhang, Miao-Miao Tao, Yun-Yi Deng, Lin-Bao Ge, Chun-Lei Shan
Summary: The study found that high expression of Cofilin-1 after cerebral ischemia-reperfusion correlates with motor function deficits, suggesting that Cofilin-1 may partially affect motor cortex function.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Dennis van der Meer, Tobias Kaufmann, Alexey A. Shadrin, Carolina Makowski, Oleksandr Frei, Daniel Roelfs, Jennifer Monereo-Sanchez, David E. J. Linden, Jaroslav Rokicki, Dag Alnaes, Christiaan de Leeuw, Wesley K. Thompson, Robert Loughnan, Chun Chieh Fan, Lars T. Westlye, Ole A. Andreassen, Anders M. Dale
Summary: The folding of the human cerebral cortex is a highly genetically regulated process that allows for a larger surface area and optimized functional organization. Sulcal depth, a measure of localized folding, has been associated with neurodevelopmental disorders. This study, the first genome-wide association study of sulcal depth, identified multiple significant loci and highlighted genetic overlap with cortical thickness and surface area, suggesting potential insights into cortical morphology.
Article
Neurosciences
Rachael F. Ryner, Isabel D. Derera, Moritz Armbruster, Anar Kansara, Mary E. Sommer, Antonella Pirone, Farzad Noubary, Michele Jacob, Chris G. Dulla
Summary: Infantile and epileptic spasms syndrome (IESS) is characterized by spasms, abnormal EEG, and epilepsy. The molecular and circuit-level causes of IESS are not well understood. This study found that inhibitory circuit dysfunction occurs in mice with deletions in adenomatous polyposis coli (APC), which may contribute to IESS-related phenotypes.
JOURNAL OF NEUROSCIENCE
(2023)
Article
Multidisciplinary Sciences
Mykhailo Y. Batiuk, Teadora Tyler, Katarina Dragicevic, Shenglin Mei, Rasmus Rydbirk, Viktor Petukhov, Ruslan Deviatiiarov, Dora Sedmak, Erzsebet Frank, Virginia Feher, Nikola Habek, Qiwen Hu, Anna Igolkina, Lilla Roszik, Ulrich Pfisterer, Diego Garcia-Gonzalez, Zdravko Petanjek, Istvan Adorjan, Peter V. Kharchenko, Konstantin Khodosevich
Summary: Schizophrenia is a widespread and complex mental disorder characterized by a reduction in abundance of GABAergic neurons and an increase in principal neurons, especially in the upper cortical layers. Many neuronal subtypes show extensive transcriptomic changes, particularly in upper-layer GABAergic neurons. Transcription factor network analysis reveals the developmental origin of these transcriptomic changes.
Article
Multidisciplinary Sciences
Ekaterina L. Ivanova, Johan G. Gilet, Vadym Sulimenko, Arnaud Duchon, Gabrielle Rudolf, Karen Runge, Stephan C. Collins, Laure Asselin, Loic Broix, Nathalie Drouot, Peggy Tiiiy, Patrick Nusbaum, Alexandre Vincent, William Magnant, Valerie Skory, Marie-Christine Birling, Guillaume Pavlovic, Juliette D. Godin, Binnaz Yalcin, Yann Herault, Pavel Draber, Jamel Chelly, Maria-Victoria Hinckelmann
NATURE COMMUNICATIONS
(2019)
Review
Biochemistry & Molecular Biology
Quentin Marlier, Tine D'aes, Sebastien Verteneuil, Renaud Vandenbosch, Brigitte Malgrange
CELLULAR AND MOLECULAR LIFE SCIENCES
(2020)
Article
Multidisciplinary Sciences
Kateryna Shostak, Zheshen Jiang, Benoit Charloteaux, Alice Mayer, Yvette Habraken, Lars Tharun, Sebastian Klein, Xinyi Xu, Hong Quan Duong, Andrii Vislovukh, Pierre Close, Alexandra Florin, Florian Rambow, Jean-Christophe Marine, Reinhard Buettner, Alain Chariot
NATURE COMMUNICATIONS
(2020)
Article
Multidisciplinary Sciences
Laure Asselin, Jose Rivera Alvarez, Solveig Heide, Camille S. Bonnet, Peggy Tilly, Helene Vitet, Chantal Weber, Carlos A. Bacino, Kristin Baranano, Anna Chassevent, Amy Dameron, Laurence Faivre, Neil A. Hanchard, Sonal Mahida, Kirsty McWalter, Cyril Mignot, Caroline Nava, Agnes Rastetter, Haley Streff, Christel Thauvin-Robinet, Marjan M. Weiss, Gladys Zapata, Petra J. G. Zwijnenburg, Frederic Saudou, Christel Depienne, Christelle Golzio, Delphine Heron, Juliette D. Godin
NATURE COMMUNICATIONS
(2020)
Article
Immunology
Pierre Lemaitre, Qiang Bai, Celine Legrand, Alain Chariot, Pierre Close, Fabrice Bureau, Christophe J. Desmet
Summary: The study revealed that specific epitranscriptomic tRNA modifications, particularly Elp3, play a crucial role in T cell biology by influencing T cell cycle entry and promoting optimal TFH responses. Overactivation of the stress-responsive transcription factor Atf4 in Elp3-deficient T cells leads to impaired T cell function, while targeting Atf4 or Chop rescues TFH responses in Elp3-deficient T cells.
JOURNAL OF IMMUNOLOGY
(2021)
Article
Immunology
Adeline Rosu, Najla El Hachem, Francesca Rapino, Kevin Rouault-Pierre, Joseph Jorssen, Joan Somja, Eve Ramery, Marc Thiry, Laurent Nguyen, Maarten Jacquemyn, Dirk Daelemans, Christopher M. Adams, Dominique Bonnet, Alain Chariot, Pierre Close, Fabrice Bureau, Christophe J. Desmet
Summary: The study reveals the crucial role of tRNA anticodon modifications in hematopoiesis, with Elp3 deficiency causing bone marrow failure and activating a p53-dependent checkpoint resembling amino acid deprivation response, ultimately promoting the development of cancer.
JOURNAL OF EXPERIMENTAL MEDICINE
(2021)
Article
Multidisciplinary Sciences
Francesca Rapino, Zhaoli Zhou, Ana Maria Roncero Sanchez, Marc Joiret, Christian Seca, Najla El Hachem, Gianluca Valenti, Sara Latini, Kateryna Shostak, Liesbet Geris, Ping Li, Gang Huang, Gabriel Mazzucchelli, Dominique Baiwir, Christophe J. Desmet, Alain Chariot, Michel Georges, Pierre Close
Summary: Wobble uridine (U-34) tRNA modifications are important for decoding AA-ending codons. The U-34-codon content of mRNAs can predict changes in ribosome translation elongation, while the resulting outcome in protein expression relies on specific hydrophilic motifs-dependent protein aggregation and clearance.
NATURE COMMUNICATIONS
(2021)
Article
Biochemistry & Molecular Biology
Elizabeth Ramos-Morales, Efil Bayam, Jordi Del-Pozo-Rodriguez, Thalia Salinas-Giege, Martin Marek, Peggy Tilly, Philippe Wolff, Edouard Troesch, Eric Ennifar, Laurence Drouard, Juliette D. Godin, Christophe Romier
Summary: This study provides insights into the molecular mechanisms of tRNA deamination by ADAT2 and the importance of the ADAT3 N-terminal domain in tRNA binding and deamination. The rotation of the N-terminal domain with respect to the catalytic domain plays a crucial role in presenting and positioning the tRNA anticodon-stem-loop correctly in the ADAT2 active site. A founder mutation in the ADAT3 N-terminal domain, causing intellectual disability, is shown to hinder optimal presentation of the tRNA anticodon-stem-loop to ADAT2.
NUCLEIC ACIDS RESEARCH
(2021)
Article
Neurosciences
Valerie Dion, Nathalie Schumacher, Nathalie Masar, Alexandra Pieltain, Pierre Tocquin, Pierre Lesoinne, Brigitte Malgrange, Renaud Vandenbosch, Rachelle Franzen
Summary: This study investigated the role of Cyclin-dependent kinase 7 (Cdk7) in oligodendrocyte differentiation. The results showed that the loss of Cdk7 led to a decrease in axonal diameter, thinner myelin sheaths, and reduced levels of major myelin-associated proteins during myelination. These findings reveal novel functions for Cdk7 in central nervous system myelination.
Article
Genetics & Heredity
Dhanya Lakshmi Narayanan, Jose Rivera Alvarez, Peggy Tilly, Michelle C. do Rosario, Vivekananda Bhat, Juliette D. Godin, Anju Shukla
Summary: Variant in the KIF21B gene is associated with neurodevelopmental disorders, leading to intellectual disability and brain malformations. This study reports a case of a 9-year-old boy presenting with delayed speech, hyperactivity, poor social interaction, and autistic features. In vivo functional analysis confirmed the pathogenicity of the novel rare variant in KIF21B.
JOURNAL OF HUMAN GENETICS
(2022)
Article
Audiology & Speech-Language Pathology
Ronald Pouyo, Keshi Chung, Laurence Delacroix, Brigitte Malgrange
Summary: Post-translational modifications, such as protein ubiquitination, play essential roles in the development and function of the inner ear. Impairment of the ubiquitin-proteasome system (UPS) has been linked to altered cochlear development and hearing loss. This review provides insights into the mechanisms of UPS and its confirmed role in normal hearing and deafness, as well as potential therapeutic strategies for UPS-associated hearing loss.
Article
Biochemistry & Molecular Biology
Tine D'aes, Quentin Marlier, Sebastien Verteneuil, Pascale Quatresooz, Renaud Vandenbosch, Brigitte Malgrange
Summary: This study investigated the role of Cdk7 in ischemic neuronal death using the OGD model. The results showed that invalidation of Cdk7 did not provide neuroprotection and no evidence of apoptosis was found in the OGD model. Neurons exposed to OGD seemed predisposed to die in an NMDA receptor-dependent manner. Due to uncertainties about cell death after OGD, caution is warranted when using this model for identifying new stroke therapies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Cell Biology
Ilyas Chohra, Keshi Chung, Subhajit Giri, Brigitte Malgrange
Summary: During transcription, DNA replication and repair, ATP-dependent chromatin remodeling complexes modify chromatin structure through repositioning and rearranging nucleosomes using the energy of ATP hydrolysis. These complexes are crucial in regulating development and stem cell biology in various organs, including the inner ear, and mutations in genes coding for their protein components have been implicated in neurosensory deafness. This review discusses the composition, structure, and functional activity of ATP-dependent chromatin remodeling complexes and their contribution to hearing and neurosensory deafness.
Article
Biochemistry & Molecular Biology
Ilyas Chohra, Subhajit Giri, Brigitte Malgrange
Summary: Researchers have generated a homozygous CHD4G1003D hESC line (WAe025-A-1) using CRISPR/eCas9-based gene editing in the WA-25 hESC line. The edited hESC line maintains a normal karyotype, pluripotency, and the ability to differentiate into three germ layers. This cell line will serve as a valuable resource for studying the functional role of CHD4 in the development and disease modeling of SIHIWES.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Cell Biology
Mona Veithen, Aurelia Huyghe, Priscilla van den Ackerveken, So-ichiro Fukada, Hiroki Kokubo, Ingrid Breuskin, Laurent Nguyen, Laurence Delacroix, Brigitte Malgrange
Summary: The transcription factor Sox9 plays a crucial role in cell fate determination in the cochlea, with its expression becoming restricted to supporting cells during hair cell differentiation. Sox9 inhibits the transcriptional activity of Atoh1, a key factor for hair cell formation, by upregulating Hey1 and HeyL antagonists. Suppression of Sox9 in sensory progenitors leads to a modest increase in hair cell number, but this is not sufficient to induce supernumerary hair cell production in vivo.
