Article
Cell Biology
Naseem Amirmokhtari, Brian D. Foresi, Shiv S. Dewan, Rachida A. Bouhenni, Matthew A. Smith
Summary: Mutations in the Cyp1b1 gene are common genetic predispositions associated with various human glaucomas, particularly primary congenital glaucoma. The role of Cyp1b1 in normal retinal projection development and its influence on retinal ganglion cell structure and function under stress conditions such as elevated ocular pressure remain largely unknown.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Neurosciences
Sonam Dolma, Abhijeet Joshi
Summary: The conduction of action potential along the axon relies heavily on the interaction between the axon and myelin-producing glial cells. Myelin, formed by Schwann cells in the peripheral nervous system and oligodendrocytes in the central nervous system, serves as insulation and facilitates action potential. The nodes of Ranvier, intermittent gaps along the myelin sheath, play a crucial role in this process. Research has identified a comprehensive proteome at the nodes of Ranvier and highlighted the importance of axon-glia interactions in various neurological disorders. This review provides an update on the molecular composition of the nodes of Ranvier and discusses the consequences of disrupted axon-glia interactions in CNS and PNS disorders.
JOURNAL OF NEUROIMMUNE PHARMACOLOGY
(2023)
Article
Clinical Neurology
Hailong Song, Przemyslaw P. McEwan, Kamar E. Ameen-Ali, Alexandra Tomasevich, Claire Kennedy-Dietrich, Alexander Palma, Edgardo J. Arroyo, Jean-Pierre Dolle, Victoria E. Johnson, William Stewart, Douglas H. Smith
Summary: This study investigated the pathophysiological changes of concussion, revealing that selective damage to white matter axons disrupts brain network connectivity and function. The integrity changes of voltage-gated sodium channels and their anchoring proteins at the nodes of Ranvier were examined. Similar changes were observed in human brain tissue, suggesting the importance of these disruptions in causing brain network dysfunction after concussion.
ACTA NEUROPATHOLOGICA
(2022)
Review
Cell Biology
Haruki Koike, Masahisa Katsuno
Summary: Myelin phagocytosis by macrophages is a key feature of demyelinating diseases, with the discovery of specific autoantibodies aiding in the identification of distinct disease entities, though the underlying mechanisms remain unclear. Macrophages appear to selectively target specific sites on myelinated fibers for demyelination, indicating the importance of localized components in initiating myelin phagocytosis.
Review
Biology
Delphine Pinatel, Catherine Faivre-Sarrailh
Summary: The distribution of specific potassium channels in axons plays a crucial role in maintaining the shape and frequency of action potentials in myelinated fibers. Kv1 channels at the axon initial segment have a significant impact on spike initiation and waveform, while their role at the juxtaparanodes is not fully understood. Exposure of Kv1 channels in demyelinating neuropathy results in altered saltatory conduction.
Editorial Material
Biochemistry & Molecular Biology
Pedro Brites, Monica M. Sousa
Summary: In a mouse model, Kreher and colleagues demonstrate that neurons, not only myelinating glia, are the primary contributors to disease progression in Krabbe disease. The neuron-specific model they generated allows for investigation of the autonomous neuronal component of this disorder.
Article
Biology
Mengjing Bao, Ruth E. Doerig, Paula Maria Vazquez-Pianzola, Dirk Beuchle, Beat Suter
Summary: In this study, the glutamylation of a-tubulin isotypes in Drosophila ovaries was investigated. It was found that the C-terminal ends of alpha Tub84B/D were glutamylated, while alpha Tub67C was not. TTLL5 was identified as the protein responsible for mono- and poly-glutamylation of alpha Tub84B/D and its proper localization. This study also demonstrated the dependence of kinesin-1 distribution, as well as other cellular functions such as axonal transport and ooplasmic streaming, on TTLL5 and alpha-tubulin glutamylation.
Article
Urology & Nephrology
Domenico Santoro, Hanna Debiec, Elisa Longhitano, Massimo Torreggiani, Antonella Barreca, Elisa Vegezzi, Anna Mazzeo, Massimo Russo, Giorgina Barbara Piccoli, Antonio Toscano, Pierre Ronco
Summary: This study reports a rare case of membranous nephropathy associated with inflammatory neuropathy. Immunological and histological examinations showed co-localization of CNTN1 antigen with immune deposits, suggesting a pathophysiological link between inflammatory neuropathies and MN.
AMERICAN JOURNAL OF KIDNEY DISEASES
(2022)
Article
Neurosciences
Anna-Lena Saur, Franziska Froeb, Matthias Weider, Michael Wegner
Summary: The transcription factor Sox10 plays a crucial role in regulation of genes related to myelin sheath components and lipid metabolism enzymes in both central and peripheral nervous systems. Our study identified a strong influence of Sox10 on adhesion-related genes in Schwann cells, working in cooperation with Krox20 to ensure coordinated formation of myelin sheath components and nodes of Ranvier during peripheral myelination.
Review
Neurosciences
Jurgen R. Schwarz
Summary: In myelinated nerve fibres, action potentials are generated at nodes of Ranvier with high density of Na+ channels and K+-selective leakage channels. Recently discovered K2P channels, which can be activated by temperature, mechanical stretch or arachidonic acid, play a crucial role in the functions of nodal K2P channels.
JOURNAL OF PHYSIOLOGY-LONDON
(2021)
Review
Clinical Neurology
Luis Querol, Emilien Delmont, Cinta Lleixa
Summary: The nodes of Ranvier (NoR) are crucial for nerve conduction and their disruption plays a significant role in immune-mediated neuropathies. Understanding the immune mechanisms affecting these specialized nodal regions has led to the classification of autoimmune nodopathies, which are defined by the site of autoimmune attack. Autoantibodies against molecules of the nodal region, paranodal demyelination induced by macrophages, and phenotypic changes of Schwann cells' nodal domains are identified as key mechanisms in the pathogenesis of autoimmune neuropathies. This review explores the autoimmune vulnerability of NoR and the underlying mechanisms leading to dysfunction in different autoimmune disorders.
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
(2023)
Article
Cell Biology
Heather Tsong, Erika L. F. Holzbaur, Andrea K. H. Stavoe
Summary: Misregulation of neuronal autophagy is involved in age-related neurodegenerative diseases. Aging leads to a decrease in autophagosome formation rate and density along the axon, but an increase in autophagic vesicle maturation. Late aging results in a significant loss of acidified vesicles in the distal axon, while the efficient transport of autophagic vesicles in both proximal and distal axons is maintained during early aging. These findings suggest that aging differentially impacts distinct aspects of neuronal autophagy.
Article
Cell Biology
Thomas J. Krzystek, Joseph A. White, Rasika Rathnayake, Layne Thurston, Hayley Hoffmar-Glennon, Yichen Li, Shermali Gunawardena
Summary: This study reveals a novel role for HTT in the retrograde movement of a Rab7-LAMP1-containing signaling late endosome and identifies adaptors Hip1 and Rilpl as important factors in this process.
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)
Article
Anatomy & Morphology
Rhona McGonigal, Hugh J. Willison
Summary: Gangliosides, a family of glycosphingolipids rich in sialic acid, play a crucial role in modulating nervous system integrity and serving as receptors in the vertebrate nervous system. Studies using transgenic mice with transferase deficiencies have helped elucidate the role of gangliosides at the node of Ranvier.
JOURNAL OF ANATOMY
(2022)
Article
Clinical Neurology
Maren Lindner, Katja Thuemmler, Ariel Arthur, Sarah Brunner, Christina Elliott, Daniel McElroy, Hema Mohan, Anna Williams, Julia M. Edgar, Cornelia Schuh, Christine Stadelmann, Susan C. Barnett, Hans Lassmann, Steve Muecklisch, Manikhandan Mudaliar, Nicole Schaeren-Wiemers, Edgar Meinl, Christopher Linington
Article
Neurosciences
Sandra Goebbels, Georg L. Wieser, Alexander Pieper, Sonia Spitzer, Bettina Weege, Kuo Yan, Julia M. Edgar, Oleksandr Yagensky, Sven P. Wichert, Amit Agarwal, Khalad Karram, Nicolas Renier, Marc Tessier-Lavigne, Moritz J. Rossner, Ragnhildur Thora Karadottir, Klaus-Armin Nave
NATURE NEUROSCIENCE
(2017)
Article
Neurosciences
Noha G. Bahey, Kamal K. E. Gadalla, Rhona Mcgonigal, Mark E. S. Bailey, Julia M. Edgar, Stuart R. Cobb
Article
Multidisciplinary Sciences
Stefan A. Berghoff, Nina Gerndt, Jan Winchenbach, Sina K. Stumpf, Leon Hosang, Francesca Odoardi, Torben Ruhwedel, Carolin Boehler, Benoit Barrette, Ruth Stassart, David Liebetanz, Payam Dibaj, Wiebke Moebius, Julia M. Edgar, Gesine Saher
NATURE COMMUNICATIONS
(2017)
Article
Neurosciences
Stephanie L. Cumberworth, Jennifer A. Barrie, Madeleine E. Cunningham, Daniely Paulino Gomes de Figueiredo, Verena Schultz, Adrian J. Wilder-Smith, Benjamin Brennan, Lindomar J. Pena, Rafael Freitas de Oliveira Franca, Christopher Linington, Susan C. Barnett, Hugh J. Willison, Alain Kohl, Julia M. Edgar
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2017)
Review
Neurosciences
Ruth M. Stassart, Wiebke Mobius, Klaus-Armin Nave, Julia M. Edgar
FRONTIERS IN NEUROSCIENCE
(2018)
Article
Biochemistry & Molecular Biology
Nicolas Snaidero, Wiebke Moebus, Tim Czopka, Liesbeth H. P. Hekking, Cliff Mathisen, Dick Verkleij, Sandra Goebbels, Julia Edgar, Doron Merkler, David A. Lyons, Klaus-Armin Nave, Mikael Simons
Article
Oncology
Ruth F. Deighton, Thierry Le Bihan, Sarah F. Martin, Alice M. J. Gerth, Mailis McCulloch, Julia M. Edgar, Lorraine E. Kerr, Ian R. Whittle, James McCulloch
JOURNAL OF NEURO-ONCOLOGY
(2014)
Article
Neurosciences
Denggao Yao, Rhona McGonigal, Jennifer A. Barrie, Joanna Cappell, Madeleine E. Cunningham, Gavin R. Meehan, Simon N. Fewou, Julia M. Edgar, Edward Rowan, Yuhsuke Ohmi, Keiko Furukawa, Koichi Furukawa, Peter J. Brophy, Hugh J. Willison
JOURNAL OF NEUROSCIENCE
(2014)
Article
Clinical Neurology
Fredrik Gruenenfelder, Mark McLaughlin, Ian R. Griffiths, James Garbern, Gemma Thomson, Peter Kuzman, Jennifer A. Barrie, Maj-lis Mcculloch, Jacques Penderis, Ruth Stassart, Klaus-Armin Nave, Julia M. Edgar
Article
Multidisciplinary Sciences
Heather Hulme, Lynsey M. Meikle, Nicole Strittmatter, Justin J. J. van der Hooft, John Swales, Ryan A. Bragg, Victor H. Villar, Michael J. Ormsby, Stephanie Barnes, Sheila L. Brown, Alex Dexter, Maya T. Kamat, Jasper C. Komen, Daniel Walker, Simon Milling, Emily K. Osterweil, Andrew S. MacDonald, Chris J. Schofield, Saverio Tardito, Josephine Bunch, Gillian Douce, Julia M. Edgar, Ruangelie Edrada-Ebel, Richard J. A. Goodwin, Richard Burchmore, Daniel M. Wall
Article
Neurosciences
Verena Schultz, Jennifer A. Barrie, Claire L. Donald, Colin L. Crawford, Margaret Mullin, Thomas J. Anderson, Tom Solomon, Susan C. Barnett, Christopher Linington, Alain Kohl, Hugh J. Willison, Julia M. Edgar
Summary: The study showed that ZIKV infection can directly impact oligodendrocytes in mice, leading to reduced density of oligodendrocytes and increased microglial reactivity. This may help explain neurodevelopmental delays in infants who appeared asymptomatic at birth.
Article
Anatomy & Morphology
Julia M. Edgar, Eleanor McGowan, Katie J. Chapple, Wiebke Mobius, Leandro Lemgruber, Robert H. Insall, Klaus-Armin Nave, Anne Boullerne
Summary: The review discusses the original contributions of Pio del Rio-Hortega to the understanding of the myelinic channel system and proposes a slightly revised model of the sheath. Research reveals the complexity of the myelinic channel system in mature myelin sheaths and its potential for subtle shape changes over time.
JOURNAL OF ANATOMY
(2021)
Article
Neurosciences
Anna M. Steyer, Tobias J. Buscham, Charlotta Lorenz, Sophie Huemmert, Maria A. Eichel-Vogel, Leonie C. Schadt, Julia M. Edgar, Sarah Koester, Wiebke Moebius, Klaus-Armin Nave, Hauke B. Werner
Summary: Healthy CNS myelin sheaths facilitate normal axonal diameter and shape, which are impaired when structural myelin proteins PLP or MAG are lacking, leading to axonal pathology and myelin outfoldings.
Article
Neurosciences
Erik Schaeffner, Mar Bosch-Queralt, Julia M. Edgar, Maria Lehning, Judith Strauss, Niko Fleischer, Theresa Kungl, Peter Wieghofer, Stefan A. Berghoff, Tilo Reinert, Martin Krueger, Markus Morawski, Wiebke Moebius, Alonso Barrantes-Freer, Jens Stieler, Ting Sun, Gesine Saher, Markus H. Schwab, Christoph Wrede, Maximilian Frosch, Marco Prinz, Daniel S. Reich, Alexander Fluegel, Christine Stadelmann, Robert Fledrich, Klaus-Armin Nave, Ruth M. Stassart
Summary: This article examines cases of multiple sclerosis and autoimmune encephalomyelitis, and finds that myelination actually accelerates axonal degeneration in an autoimmune environment, challenging the belief that myelin is protective for axons.
NATURE NEUROSCIENCE
(2023)
