Review
Biochemistry & Molecular Biology
Reem Abouward, Giampietro Schiavo
Summary: Localisation of mRNA plays a crucial role in establishing and maintaining cellular polarity, synaptic plasticity, and other biological processes. Recent discoveries regarding RNA hitchhiking on cytoplasmic organelles have significant implications on our understanding of neuronal function in development, adulthood, and neurodegeneration. The mechanisms controlling mRNA localisation are largely conserved across evolution and have been extensively studied in various biological models.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Mariana Santana Dias, Xiaoyue Luo, Vinicius Toledo Ribas, Hilda Petrs-Silva, Jan Christoph Koch
Summary: Glaucoma is a neurodegenerative disease that leads to progressive vision loss by affecting the retinal ganglion cells (RGCs). Impairment of axonal transport at the optic nerve head (ONH) is an important feature of glaucoma, and is believed to occur early in the disease before axonal and somal degeneration. Understanding the role of axonal transport in glaucoma is crucial for deciphering disease mechanisms and developing early therapies to prevent irreversible neuronal damage.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Anu Antony, Neville Ng, Antonio Lauto, Jens R. Coorssen, Simon J. Myers
Summary: This study elucidated the crucial role of Ca2+-activated protease calpain in HSN1A, by regulating cellular cytoskeleton stability and microtubule transport function.
DNA AND CELL BIOLOGY
(2022)
Article
Neurosciences
R. Mabrouk, P. O. Miettinen, H. Tanila
Summary: Understanding how dystrophic neurites form around amyloid plaques is crucial for early pathophysiology of Alzheimer's disease. Three prevailing hypotheses are: (1) dystrophies result from extracellular amyloid-beta (A beta) toxicity; (2) dystrophies result from accumulation of A beta into distal neurites; and (3) dystrophies represent blebbing of the somatic membrane of a neuron with high A beta load. By utilizing a unique feature of the 5xFAD AD mouse model, the study provides evidence supporting hypothesis 2 as the most likely mechanism of dystrophic neurite formation.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Cell Biology
Cara R. Schiavon, Gerald S. Shadel, Uri Manor
Summary: CMT disease is a progressive, inherited neurological disorder associated with mutations in at least 80 different genes. Clinical manifestations typically involve peripheral neurons, with some mutations potentially leading to mitochondrial mobility defects, suggesting a common underlying disease mechanism.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Immunology
Giuseppina Tesco, Selene Lomoio
Summary: Neurodegenerative diseases are characterized by the loss of specific neuronal populations and are associated with aging. Axonal transport and neuroinflammation play important roles in the pathogenesis of these diseases. However, the causal relationship between these mechanisms and neuronal degeneration is still uncertain. This article reviews the latest evidence on the interplay between axonal transport perturbations and CNS inflammation in neurodegenerative diseases, their potential as therapeutic targets, and the need for early biomarkers.
SEMINARS IN IMMUNOLOGY
(2022)
Article
Neurosciences
Rachel T. Clements, Lauren E. Fuller, Kyle R. Kraemer, Samantha A. Radomski, Sarah Hunter-Chang, Wesley C. Hall, Alborz A. Kalantar, Bradley R. Kraemer
Summary: This study introduces an improved method for analyzing neurite degeneration, enhancing accuracy through selection criteria modification, image artifact removal, and contrast optimization. The ImageJ macro allows rapid and automated DI analysis, expanding the application to experiments involving dissociated cultures and demonstrating increased accuracy and efficiency compared to conventional methods. Additionally, the method is applied to investigate neurite degeneration in a PD cell culture model, potentially providing new insights into neurodegenerative disorders.
Article
Geriatrics & Gerontology
Xiaoman Yang, Jialing Wang, Weiqi Zeng, Xiaoqian Zhang, Xiaomei Yang, Yu Xu, Yan Xu, Xuebing Cao
Summary: This study introduces a novel rat model of Parkinson's disease characterized by widespread, seed-initiated endogenous alpha-Syn pathology, impaired axonal transport, and a neurodegenerative cascade in the nigrostriatal system. This model provides an important platform for further research on specific pathogenic mechanisms and pre-clinical evaluations of novel therapeutic strategies.
FRONTIERS IN AGING NEUROSCIENCE
(2022)
Article
Neurosciences
Benjamin Combs, Kyle R. Christensen, Collin Richards, Andrew Kneynsberg, Rebecca L. Mueller, Sarah L. Morris, Gerardo A. Morfini, Scott T. Brady, Nicholas M. Kanaan
Summary: The study demonstrates that FTLD mutant tau proteins, P301L and R5L, can elicit a toxic effect on axonal transport as monomeric proteins. This suggests a mechanism of tau toxicity involving aberrant activation of a specific PP1 gamma-dependent pathway that disrupts axonal transport in neurons.
JOURNAL OF NEUROSCIENCE
(2021)
Article
Geriatrics & Gerontology
Md Golam Sharoar, John Zhou, Marc Benoit, Wanxia He, Riqiang Yan
Summary: The formation of RIDNs, composed of abnormally clustered tubular ER and degenerating mitochondria, occurs early in the growth of amyloid plaques in AD brains. DCTN6 deficiency enhances RTN3 protein levels and hippocampus-specific RIDN formation in aging brains of transgenic mice. The DCTN6-RTN3 interaction mediates tubular ER trafficking in axons, and deficiency in DCTN6 impairs axonal ER trafficking, leading to abnormal ER accumulation and RIDN formation in aging mouse brains.
NEUROBIOLOGY OF AGING
(2021)
Article
Neurosciences
Flavia Malvestio Sisti, Neife Aparecida Guinaim dos Santos, Lilian do Amaral, Antonio Cardozo dos Santos
Summary: Carvacrol (CARV) is a phytochemical with promising neurotrophic effects in neuronal models, potentially beneficial in treating neurodegenerative and neurological diseases by inducing neurite outgrowth and enhancing neuronal plasticity and bioenergetics.
NEUROTOXICITY RESEARCH
(2021)
Review
Biochemistry & Molecular Biology
Bohm Lee, Yongcheol Cho
Summary: Neurons in different nervous systems have varying abilities to regenerate, with rodents serving as key models for studying these mechanisms and advancing understanding of human diseases and treatment methods.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Neurosciences
Jessica L. Wickline, Sabrina Smith, Riley Shin, Kristian Odfalk, Jesse Sanchez, Martin Javors, Brett Ginsburg, Sarah C. Hopp
Summary: Epidemiological studies suggest that L-type calcium channel antagonists may reduce the incidence of age-associated neurodegenerative diseases, including Alzheimer's disease. However, the protective mechanism of LTCC antagonists is unknown.
Article
Biochemistry & Molecular Biology
Yubing Lu, Hisashi Fujioka, Wenzhang Wang, Xiongwei Zhu
Summary: Mitochondrial dysfunction plays a key role in the development of AD, and bezafibrate can improve mitochondrial function, alleviate neuroinflammation, and enhance cognitive functions.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2023)
Editorial Material
Cell Biology
Joseph C. Roney, Sunan Li, Tamar Farfel-Becker, Ning Huang, Tao Sun, Yuxiang Xie, Xiu-Tang Cheng, Mei-Yao Lin, Frances M. Platt, Zu-Hang Sheng
Summary: The study reveals an impairment of lysosome transport to distal axons in Niemann-Pick disease type C (NPC), leading to accumulation of autophagic vacuoles (AVs) at axon terminals. Elevated cholesterol on NPC lysosome membranes abnormally sequesters motor-adaptors of axonal lysosome delivery, disrupting maturation of axonal AVs during their retrograde transport. Reducing lysosomal lipid levels rescues lysosome transport into NPC axons, reducing axonal autophagic stress in early stages of NPC disease.
Article
Neurosciences
Pierre De Rossi, Virginie Buggia-Prevot, Benjamin L. L. Clayton, Jared B. Vasquez, Carson van Sanford, Robert J. Andrew, Ruben Lesnick, Alexandra Botte, Carole Deyts, Someya Salem, Eshaan Rao, Richard C. Rice, Angele Parent, Satyabrata Kar, Brian Popko, Peter Pytel, Steven Estus, Gopal Thinakaran
MOLECULAR NEURODEGENERATION
(2016)
Article
Biochemistry & Molecular Biology
P. De Rossi, E. Harde, J. P. Dupuis, L. Martin, N. Chounlamountri, M. Bardin, C. Watrin, C. Benetollo, K. Pernet-Gallay, H. J. Luhmann, J. Honnorat, G. Malleret, L. Groc, A. Acker-Palmer, P. A. Salin, C. Meissirel
MOLECULAR PSYCHIATRY
(2016)
Article
Clinical Neurology
Lenka Mikasova, Pierre De Rossi, Delphine Bouchet, Francois Georges, Veronique Rogemond, Adrien Didelot, Claire Meissirel, Jerome Honnorat, Laurent Groc
Article
Multidisciplinary Sciences
Claire Meissirel, Carmen Ruiz de Almodovar, Ellen Knevels, Cathy Coulon, Naura Chounlamountri, Inmaculada Segura, Pierre de Rossi, Stefan Vinckier, Kristof Anthonis, Berangere Deleglise, Maria de Mol, Carine Ali, Karel Dassonville, Ellen Loyens, Jerome Honnorat, Yvette Michotte, Veronique Rogemond, Ilse Smolders, Thomas Voets, Denis Vivien, Pieter Vanden Berghe, Ludo Van Den Bosch, Wim Robberecht, Alain Chedotal, Salvatore Oliviero, Mieke Dewerchin, Dietmar Schmucker, Nicole Thomasset, Paul Salin, Peter Carmeliet
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2011)
Article
Clinical Neurology
Pierre De Rossi, Robert J. Andrew, Timothy F. Musial, Virginie Buggia-Prevot, Guilian Xu, Moorthi Ponnusamy, Han Ly, Sofia V. Krause, Richard C. Rice, Valentine de l'Estoile, Tess Valin, Someya Salem, Florin Despa, David R. Borchelt, Vytas P. Bindokas, Daniel A. Nicholson, Gopal Thinakaran
Article
Biochemistry & Molecular Biology
Robert J. Andrew, Pierre De Rossi, Phuong Nguyen, Haley R. Kowalski, Aleksandra J. Recupero, Thomas Guerbette, Sofia V. Krause, Richard C. Rice, Lisa Laury-Kleintop, Steven L. Wagner, Gopal Thinakaran
JOURNAL OF BIOLOGICAL CHEMISTRY
(2019)
Article
Cell Biology
Pierre De Rossi, Toshihiro Nomura, Robert J. Andrew, Nicolas Y. Masse, Vandana Sampathkumar, Timothy F. Musial, Ari Sudwarts, Aleksandra J. Recupero, Thomas Le Metayer, Mitchell T. Hansen, Ha-Na Shim, Sofia V. Krause, David J. Freedman, Vytas P. Bindokas, Narayanan Kasthuri, Daniel A. Nicholson, Anis Contractor, Gopal Thinakaran
Review
Cell Biology
Aurelie Zbinden, Manuela Perez-Berlanga, Pierre De Rossi, Magdalini Polymenidou
DEVELOPMENTAL CELL
(2020)
Article
Medicine, Research & Experimental
Marc Emmenegger, Elena De Cecco, Marian Hruska-Plochan, Timo Eninger, Matthias M. Schneider, Melanie Barth, Elena Tantardini, Pierre de Rossi, Mehtap Bacioglu, Rebekah G. Langston, Alice Kaganovich, Nora Bengoa-Vergniory, Andres Gonzalez-Guerra, Merve Avar, Daniel Heinzer, Regina Reimann, Lisa M. Haesler, Therese W. Herling, Naunehal S. Matharu, Natalie Landeck, Kelvin Luk, Ronald Melki, Philipp J. Kahle, Simone Hornemann, Tuomas P. J. Knowles, Mark R. Cookson, Magdalini Polymenidou, Mathias Jucker, Adriano Aguzzi
Summary: The expression pattern of LAG3 in human and mouse brains was analyzed, with no evidence of neurons expressing LAG3. While LAG3 was confirmed to interact with alpha-synuclein fibrils, the specificity of this interaction appears limited. Additionally, overexpression of LAG3 did not worsen alpha-synuclein pathology.
EMBO MOLECULAR MEDICINE
(2021)
Article
Multidisciplinary Sciences
Jelena Scekic-Zahirovic, Inmaculada Sanjuan-Ruiz, Vanessa Kan, Salim Megat, Pierre De Rossi, Stephane Dieterle, Raphaelle Cassel, Marguerite Jamet, Pascal Kessler, Diana Wiesner, Laura Tzeplaeff, Valerie Demais, Sonu Sahadevan, Katharina M. Hembach, Hans-Peter Muller, Gina Picchiarelli, Nibha Mishra, Stefano Antonucci, Sylvie Dirrig-Grosch, Jan Kassubek, Volker Rasche, Albert Ludolph, Anne-Laurence Boutillier, Francesco Roselli, Magdalini Polymenidou, Clotilde Lagier-Tourenne, Sabine Liebscher, Luc Dupuis
Summary: Mutations in the RNA binding protein FUS are associated with ALS. Here the authors show that in FUS knock-in mice there is a progressive increase in neuronal activity in the frontal cortex which is associated with altered synaptic gene expression.
NATURE COMMUNICATIONS
(2021)
Article
Multidisciplinary Sciences
Sonu Sahadevan, Katharina M. Hembach, Elena Tantardini, Manuela Perez-Berlanga, Marian Hruska-Plochan, Salim Megat, Julien Weber, Petra Schwarz, Luc Dupuis, Mark D. Robinson, Pierre De Rossi, Magdalini Polymenidou
Summary: Mutations in the RNA-binding protein FUS disrupt its nuclear localization and contribute to amyotrophic lateral sclerosis. Accumulation of synaptic FUS in early disease leads to synaptic impairment, potentially representing an initial trigger of neurodegeneration. The study identifies proteins associated with synapse organization and plasticity that are differentially regulated in an ALS mouse model.
NATURE COMMUNICATIONS
(2021)
Article
Biochemistry & Molecular Biology
Pierre De Rossi, Amanda J. Lewis, Johanna Furrer, Laura De Vos, Tomas Demeter, Aurelie Zbinden, Weijia Zhong, Vera Wiersma, Carlo Scialo, Julien Weber, Zhongning Guo, Stefano Scaramuzza, Marta Di Fabrizio, Carolin Boeing, Daniel Castano-Diez, Ashraf Al-Amoudi, Manuela Perez-Berlanga, Tammaryn Lashley, Henning Stahlberg, Magdalini Polymenidou
Summary: Pathological TDP-43 follows a prion-like cascade, but the molecular determinants of this process remain unknown. Different subtypes demonstrate distinct amplification efficiency and timeline when inoculated into cells. The resulting neoaggregates also exhibit subtype-specific characteristics, reflecting the diversity seen in patient pathology.