Article
Clinical Neurology
Yasuo Miki, Kosuke Kamata, Shintaro Goto, Hirotake Sakuraba, Fumiaki Mori, Kazufumi Yamagata, Hiroshi Kijima, Shinsaku Fukuda, Koichi Wakabayashi
Summary: In adult neuronal intranuclear inclusion disease (NIID), both neurons and glial cells harbor intranuclear inclusions, which can also be found in the peripheral autonomic nervous system, visceral organs, and skin. This study reports a case of NIID in a 78-year-old Japanese male who exhibited mild tremors but did not show typical radiographic abnormalities. Pathological analysis revealed that neuronal intranuclear inclusions were particularly frequent in the hippocampal formation, while glial intranuclear inclusions were barely evident in the affected regions. The case also had an immunohistochemical profile differing from that of typical adult NIID.
Article
Multidisciplinary Sciences
Sefan Asamitsu, Yasushi Yabuki, Susumu Ikenoshita, Kosuke Kawakubo, Moe Kawasaki, Shingo Usuki, Yuji Nakayama, Kaori Adachi, Hiroyuki Kugoh, Kazuhiro Ishii, Tohru Matsuura, Eiji Nanba, Hiroshi Sugiyama, Kohji Fukunaga, Norifumi Shioda
Summary: The study explores the pathogenesis and potential treatment of FXTAS, proposing a novel therapeutic strategy targeting G4RNA prionoids by inhibiting RAN translation and abnormal protein aggregation induced by CGG-G4RNA.
Article
Cell Biology
Masato Uemura, Stefan Blankvoort, Sean Shui Liang Tok, Li Yuan, Luis Fernando Cobar, Kwok Keung Lit, Ayumu Tashiro
Summary: The distribution of neurogenesis in the dentate gyrus is positionally associated with active excitatory neurons and dissociated from a type of interneuron with high-burst tendency. This suggests that behaviorally relevant activity of excitatory-inhibitory neuronal circuits can define a microenvironment stimulating/inhibiting neurogenesis, contributing to strategic recruitment of new neurons for modification of functionally relevant neural circuits.
Review
Clinical Neurology
Xiang Qin, Hao Chen, Chenguang Zhou, XiaoLong Wang, Jingjing Gao, Nan Guo, Yujing Wang, Shaodong Li
Summary: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease characterized by the presence of eosinophilic intranuclear inclusions in the nervous system and multiple visceral organs. Adult-onset NIID patients often present with symptoms like headache, dizziness, and limb weakness, with characteristic high intensity signal changes in brain imaging. Skin biopsy pathology combined with genetic testing can aid in accurate diagnosis.
NEUROLOGICAL SCIENCES
(2021)
Article
Multidisciplinary Sciences
Aiste Vitkunaite, Aida Laurinaviciene, Benoit Plancoulaine, Allan Rasmusson, Richard Levenson, Michael Shribak, Arvydas Laurinavicius
Summary: The study provides evidence that intranuclear birefringent inclusions (IBI) are caused by liquid paraffin-macromolecular crystals formed during paraffin-embedding procedures within cells, potentially reflecting an active transcriptional status.
SCIENTIFIC REPORTS
(2021)
Article
Neurosciences
Joseph Cichon, Andrzej Z. Wasilczuk, Loren L. Looger, Diego Contreras, Max B. Kelz, Alex Proekt
Summary: This study demonstrates that ketamine can cause a switch in the active population of excitatory neurons in the cortical layers, which leads to impairments in sensory processing and dissociative-like state. These findings provide insights into how the brain can become disconnected from the environment while maintaining internal subjective experiences.
NATURE NEUROSCIENCE
(2023)
Article
Cell Biology
Maijia Liao, Alex D. Bird, Hermann Cuntz, Jonathon Howard
Summary: This study found that the subtree size distribution of neurons follows power laws, indicating scale invariance as a characteristic of optimal morphology.
Review
Multidisciplinary Sciences
Liqun Luo
Summary: While individual neurons are the building blocks of the nervous system, they function by collaborating in neuronal circuits with specific patterns of synaptic connectivity. Understanding how these circuits assemble and evolve can help bridge the gap between individual neuron biology and brain function, leading to insights into neural computations and behavior.
Article
Clinical Neurology
Yuwen Cao, Wotu Tian, Jingying Wu, Xingwang Song, Li Cao, Xinghua Luan
Summary: In this study, the methylation status of the NOTCH2NLC promoter was analyzed in NIID patients and healthy controls. The results showed that the methylation levels of GGC repeats and adjacent CpG islands were higher in the NIID patients. Furthermore, correlations between methylation levels and disease features were explored for the first time, providing new insights into the pathogenic mechanism of NIID.
JOURNAL OF NEUROLOGY
(2022)
Article
Mathematics, Applied
Jean-Philippe Thivierge, Eloise Giraud, Michael Lynn, Annie Theberge Charbonneau
Summary: This paper proposes a new class of structured reservoir models that incorporate a diversity of cell types and their known connections. Experimental results show that the model has two distinct dynamical regimes and can be used to relay and generate chaotic Lorenz attractors.
Article
Clinical Neurology
Iulian V. Podar, Daniel A. P. Gutmann, Florian Harmuth, Tobias B. Haack, Stephan Ossowski, Holger Hengel, Antje Bornemann, Ludger Schoels, Oliver Neuhaus
Summary: This case report describes a Caucasian female patient who presented with paroxysmal encephalopathy twice. Diagnosis was made using various methods including neurocranium magnetic resonance imaging, skin biopsy for histology, and long-read genome sequencing. The patient's MRI findings were consistent with typical features of adult onset neuronal intranuclear inclusion disease. Skin biopsy confirmed the presence of intranuclear inclusion bodies, and long-read genome sequencing revealed an expansion of the GGC repeat in exon 1 of NOTCH2NLC. This case highlights the possibility of adult onset NOTCH2NLC-GGC-positive NIID in non-Asian individuals.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Multidisciplinary Sciences
Ana Gonzalez-Ramos, Eliska Waloschkova, Apostolos Mikroulis, Zaal Kokaia, Johan Bengzon, Marco Ledri, My Andersson, Merab Kokaia
Summary: This study demonstrates the potential for regulating human neuronal network activity by transplanting light-sensitive GABAergic interneurons. It provides a new avenue for controlling neurological disorders through precise temporal modulation of network excitability.
SCIENTIFIC REPORTS
(2021)
Review
Cell Biology
Miho Nakajima
Summary: Adaptive behavior relies on context-dependent cognitive control supported by the prefrontal cortex (PFC). This review discusses two specific cell groups, context/brain state responsive interneuron subtypes and output decoder neurons, that may help understand the underlying circuit principles of PFC function and provide new insights into PFC dynamics.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
(2022)
Article
Cell Biology
Aidan Schneider, Mehdi Azabou, Louis McDougall-Vigier, David F. Parks, Sahara Ensley, Kiran Bhaskaran-Nair, Tomasz Nowakowski, Eva L. Dyer, Keith B. Hengen
Summary: Cell type is a crucial factor in determining the role of neurons in a circuit. This study investigates the influence of a neuron's transcriptomic type on the timing of its activity. The researchers develop a deep-learning architecture that can analyze interevent intervals across various timescales. They find that transcriptomic cell-class information is present in the timing of single neuron activity in the brain and can be universal across different stimuli.
Article
Clinical Neurology
Lu Zhou, Yun Tian, Sizhe Zhang, Bin Jiao, Xinxin Liao, Yafang Zhou, Qiao Xiao, Jin Xue, Ranhui Duan, Beisha Tang, Lu Shen
Summary: This study investigated the features of autonomic dysfunction (AutD) in patients with neuronal intranuclear inclusion disease (NIID). It found that 94.26% of patients had AutD and the severity of AutD was higher in patients compared to controls. The total SCOPA-AUT score was associated with age, disease duration, impairment of daily living ability, and psychiatric symptoms.
FRONTIERS IN NEUROLOGY
(2023)
Article
Cell Biology
Emanuela Paldino, Vincenza D'Angelo, Giuseppe Sancesario, Francesca R. Fusco
CELL DEATH DISCOVERY
(2020)
Article
Cell Biology
Emanuela Paldino, Vincenza D'Angelo, Daunia Laurenti, Cecilia Angeloni, Giuseppe Sancesario, Francesca R. Fusco
Article
Biochemistry & Molecular Biology
Vincenza D'Angelo, Mauro Giorgi, Emanuela Paldino, Silvia Cardarelli, Francesca R. Fusco, Ilaria Saverioni, Roberto Sorge, Giuseppina Martella, Stefano Biagioni, Nicola B. Mercuri, Antonio Pisani, Giuseppe Sancesario
Summary: The study found regional differences in the expression and aggregation of A2A receptors in the basal ganglia of a DYT1 mouse model of dystonia, with varying levels of expression observed in different subpopulations of neurons. Based on the different expression patterns of A2A receptors among neuronal subpopulations, it suggests that the pathophysiology of dystonia may be associated with a functional imbalance between the indirect and direct pathways.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Psychiatry
Antonella Ferrante, Zaira Boussadia, Antonella Borreca, Cinzia Mallozzi, Giorgia Pedini, Laura Pacini, Antonella Pezzola, Monica Armida, Fabrizio Vincenzi, Katia Varani, Claudia Bagni, Patrizia Popoli, Alberto Martire
Summary: In fragile X syndrome, the functional synergistic coupling between A(2A) and mGlu5 receptors may be increased. Chronic A(2A)R blockade partially restores some phenotypes of Fmr1 KO mice.
TRANSLATIONAL PSYCHIATRY
(2021)
Article
Multidisciplinary Sciences
Sara Carloni, Alice Bertocchi, Sara Mancinelli, Martina Bellini, Marco Erreni, Antonella Borreca, Daniele Braga, Silvia Giugliano, Alessandro M. Mozzarelli, Daria Manganaro, Daniel Fernandez Perez, Federico Colombo, Antonio Di Sabatino, Diego Pasini, Giuseppe Penna, Michela Matteoli, Simona Lodato, Maria Rescigno
Summary: The passage discusses the presence of psychosocial disturbances in up to 40% of patients with inflammatory bowel disease, and identifies the potential role of a deregulated gut-brain vascular axis in causing mental deficits in these individuals.
Review
Neurosciences
Stefano Farioli-Vecchioli, Valentina Ricci, Silvia Middei
Summary: This article reviews the literature on adult hippocampal neurogenesis (AHN) and AD, focusing on studies in humans and mouse models. Impaired adult neurogenesis in AD is found to be associated with memory decline. Stimulating neurogenesis has the potential to rescue memory capacities in AD.
Editorial Material
Neurosciences
Silvia Middei
Article
Biochemistry & Molecular Biology
Silvia Middei, Ludovica Giorgini, Valentina Vacca, Francesca Storri, Sabrina Putti, Georgios Strimpakos, Marcello Raspa, Ferdinando Scavizzi, Fabiola Moretti, Francesca R. D'Amato
Summary: Epidemiological evidence suggests that stress and aversive psychological conditions can influence cancer progression, while well-being can provide protection. This study used mice to demonstrate that being raised in a positive psychological environment can confer protection against tumor development. The involvement of p53 and p21 proteins may play a role in this protective effect.
Review
Biochemistry & Molecular Biology
Emanuela Paldino, Francesca Romana Fusco
Summary: Huntington's disease is a neurodegenerative disease caused by a mutation in the IT15 gene encoding huntingtin protein. Inflammation plays a significant role in neuronal death, making drugs targeting inflammation powerful tools for treating Huntington's disease.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Marco Rasile, Eliana Lauranzano, Elisa Faggiani, Margherita M. Ravanelli, Federico S. Colombo, Filippo Mirabella, Irene Corradini, Maria L. Malosio, Antonella Borreca, Elisa Focchi, Davide Pozzi, Toni Giorgino, Isabella Barajon, Michela Matteoli
Summary: This study reports that exposure of pregnant mice to an immunostimulant called Poly I:C during early fetal development can lead to abnormal brain vessels and intracerebral hemorrhages in the offspring, specifically in males. The researchers found that this prenatal infection model induces the production of TGF-beta 1, which alters the expression of certain proteins in brain endothelial cells and leads to low pericyte coverage, increasing the risk of hemorrhagic events in adult offspring. These findings provide a mechanistic framework for understanding the association between intragravidic infections and anomalies of the neural vasculature, which may contribute to neuropsychiatric disorders.
Article
Biochemistry & Molecular Biology
Emanuela Paldino, Vincenza D'angelo, Mariangela Massaro Cenere, Ezia Guatteo, Simone Barattucci, Giorgia Migliorato, Nicola Berretta, Olaf Riess, Giuseppe Sancesario, Nicola Biagio Mercuri, Francesca Romana Fusco
Summary: This study used immunohistochemical studies to investigate neuropathological changes in a rat transgenic model of Parkinson's disease (PD). It found that in the advanced stages of the disease, there was neuronal loss in the dorsolateral part of the striatum, along with a dramatic loss of parvalbumin interneurons. A marked decrease in the neurotrophic factor called glial-derived neurotrophic factor (GDNF) was also observed in the striatum and substantia nigra of these animals. This confirms the involvement of the striatum in PD pathophysiology and the importance of GDNF in maintaining the health of the substantia nigra.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Nicla Romano, Bruna Di Giacomo, Veronica Nobile, Antonella Borreca, Daniela Willems, Francesca Tilesi, Elisabetta Catalani, Manasi Agrawal, Kristy Welshhans, Sara Ricciardi, Davide Cervia, Marcello Ceci
Summary: This study reveals a mechanism by which FMRP regulates the localization of translational machinery and its translation activity of specific mRNAs by interacting with RACK1. These findings provide new insights into the altered development in Fragile X syndrome.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
Ada Ledonne, Mariangela Massaro Cenere, Emanuela Paldino, Vincenza D'Angelo, Sebastian Luca D'Addario, Nicolas Casadei, Annalisa Nobili, Nicola Berretta, Francesca R. Fusco, Rossella Ventura, Giuseppe Sancesario, Ezia Guatteo, Nicola Biagio Mercuri
Summary: Prolonged overexpression of alpha-synuclein has detrimental effects on the structural and functional properties of substantia nigra pars compacta dopamine neurons, including compromised soma and dendritic arborization, increased firing activity, and altered ion channel function.
MOVEMENT DISORDERS
(2023)
Article
Multidisciplinary Sciences
Antonella Borreca, Mariassunta De Luca, Antonella Ferrante, Zaira Boussadia, Annabella Pignataro, Alberto Martire, Martine Ammassari-Teule
Summary: Mice with deletion of the FMR1 gene show episodic memory impairments and synaptic plasticity defects. In this study, the researchers investigate how entorhinal and hippocampal synapses are remodeled when mice succeed or fail to learn in the novel object recognition paradigm. The findings reveal that the inability of mice to detect object novelty primarily stands in their impediment to elaborate sensory/perceptive object representations.
SCIENTIFIC REPORTS
(2023)
Meeting Abstract
Clinical Neurology
Giuseppe Sancesario, Vincenza D'Angelo, Giorgio Bernardi, Francesca Fusco, Giuseppina Martella, Emanuela Paldino, Silvia Cardarelli, Mauro Giorgi, Nicola Mercuri, Antonio Pisani
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2021)