Article
Biochemistry & Molecular Biology
Jianxun Yi, Ang Li, Xuejun Li, Kiho Park, Xinyu Zhou, Frank Yi, Yajuan Xiao, Dosuk Yoon, Tao Tan, Lyle W. Ostrow, Jianjie Ma, Jingsong Zhou
Summary: Respiratory failure due to progressive respiratory muscle weakness is the most common cause of death in amyotrophic lateral sclerosis (ALS). Research has shown that the muscle protein MG53 can repair NMJ damage, protect the diaphragm muscle from injury, preserve NMJ integrity, and slow the progression of ALS. This indicates MG53 may be a potentially safe and effective therapy for ALS.
Article
Cell Biology
Maria-Letizia Campanari, Anca Marian, Sorana Ciura, Edor Kabashi
Summary: The study suggests that AChE plays a crucial role in ALS pathology by regulating NMJ function. Knockdown of TDP-43 in zebrafish led to NMJ dysfunction, which was partially rescued by overexpression of human AChE. This finding provides insights into the early defects in NMJs in ALS and highlights AChE as a potential therapeutic target for the disease.
Article
Clinical Neurology
Soo Yeon Lee, Hye-Yeon Cho, Jung-Pyo Oh, Jiae Park, Sang-Hun Bae, Haesun Park, Eun Jung Kim, Ji-Hyun Lee
Summary: The combination of nebivolol and donepezil efficiently prevents the progression of ALS, benefitting the quality of life and life expectancy of patients.
Review
Medicine, General & Internal
Robert Rusina, Rik Vandenberghe, Rose Bruffaerts
Summary: ALS patients can develop cognitive and behavioral impairments, resembling frontotemporal dementia. Early detection and personalized management are crucial for improving outcomes in ALS-FTSD patients, who often have a worse prognosis compared to ALS or frontotemporal dementia alone.
Review
Neurosciences
Haibo Wang, Manohar Kodavati, Gavin W. Britz, Muralidhar L. Hegde
Summary: Neurodegenerative disorders like ALS and FTD are commonly linked to DNA damage accumulation and repair deficiency, with neurons being particularly vulnerable. Recent studies have shown that RNA/DNA binding proteins are involved in diverse DNA repair pathways.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2021)
Review
Clinical Neurology
Stephanie L. Rayner, Alison Hogan, Jennilee M. Davidson, Flora Cheng, Luan Luu, Marco Morsch, Ian Blair, Roger Chung, Albert Lee
Summary: ALS is a common disease characterized by the degeneration of motor neurons in the brain and spinal cord, and is clinically linked to FTD dementia. Identifying gene mutations associated with ALS/FTD is valuable for understanding the disease process.
Article
Behavioral Sciences
Nuno A. S. Oliveira, Brigida R. Pinho, Jorge M. A. Oliveira
Summary: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that causes disability and motor impairment. Zebrafish has emerged as a promising model for studying ALS due to its similarities to humans and the ability to study behavioral and pathophysiological phenotypes. This review discusses the relevance of zebrafish in ALS studies, model induction strategies, and various zebrafish models of ALS, highlighting their validity and potential for drug testing.
NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS
(2023)
Article
Clinical Neurology
Edoardo Nicolo Aiello, Debora Pain, Alice Radici, Kalliopi Marinou Aktipi, Riccardo Sideri, Ildebrando Appollonio, Gabriele Mora
Summary: This study found cognitive deficits in ALS patients, with classical and predominant-upper motor neuron phenotypes performing the worst. Poorer functional scores correlated with poorer cognitive scores. These findings are important for understanding the cognitive impact of ALS.
NEUROLOGICAL SCIENCES
(2022)
Review
Neurosciences
Koy Chong Ng Kee Kwong, Pratap K. Harbham, Bhuvaneish T. Selvaraj, Jenna M. Gregory, Suvankar Pal, Giles E. Hardingham, Siddharthan Chandran, Arpan R. Mehta
Summary: This review summarizes the 40-year history of CSF toxicity studies in ALS and discusses various proposed mechanisms such as glutamate excitotoxicity, proteotoxicity, and oxidative stress. The potential implications of a toxic CSF circulatory system in the pathophysiology of ALS and its significance in current ALS research are also considered.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2021)
Article
Neurosciences
Pablo Rozas, Cristina Pinto, Francisca Martinez Traub, Rodrigo Diaz, Viviana Perez, Daniela Becerra, Patricia Ojeda, Jorge Ojeda, Madison T. Wright, Jessica Mella, Lars Plate, Juan Pablo Henriquez, Claudio Hetz, Danilo B. Medinas
Summary: Overexpression of ERp57 in experimental ALS using mutant SOD1 mice delayed deterioration of electrophysiological activity and maintained muscle innervation at early-symptomatic stage, improving motor performance without affecting survival. The neuroprotective effects of ERp57 overexpression were correlated with increased levels of synaptic and actin cytoskeleton proteins in the spinal cord.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Article
Physiology
Ritika Ghosal, Gissela Borrego-Soto, Johann K. Eberhart
Summary: Forming a vertebrate head requires the integration of multiple tissue types, and prenatal alcohol exposure can cause various birth defects in the head. This study investigated the effects of ethanol on individual tissue types and their integration during craniofacial development. It was found that ethanol exposure induced ectopic cranial muscle and nerve defects, but only had slight effects on skeletal pattern. The defects in different tissues were independent of each other, and the effects of ethanol on the vertebrate head were highly pleiotropic.
FRONTIERS IN PHYSIOLOGY
(2023)
Article
Biotechnology & Applied Microbiology
Marina L. Garcia-Vaquero, Marjorie Heim, Barbara Flix, Marcelo Pereira, Lucile Palin, Tania M. Marques, Francisco R. Pinto, Javier de las Rivas, Aaron Voigt, Florence Besse, Margarida Gama-Carvalho
Summary: This study demonstrates that SMA and ALS disease-associated genes linked to RNA metabolism functionally converge on neuronal protein complexes, providing a new hypothesis to explain the common motor neuron phenotype. In addition, the study found that these functional modules are enriched in orthologues of human neuronal disease genes.
Article
Geriatrics & Gerontology
Sunghee Kim, Subin An, Jinwoo Lee, Yideul Jeong, Chang-Lim You, Hyebeen Kim, Ju-Hyeon Bae, Chae-Eun Yun, Dongryul Ryu, Gyu-Un Bae, Jong-Sun Kang
Summary: The cell adhesion molecule Cdon plays a critical role in motor neuron function and nerve repair. Mice lacking Cdon exhibit middle age onset lethality and aging-related decline in motor function. Additionally, these mice also show impairment in motor function recovery and incomplete neuromuscular junction occupation.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2023)
Article
Biochemistry & Molecular Biology
Akira Ishiguro, Akira Katayama, Akira Ishihama
Summary: The study found that TDP-43 and FUS have different target selectivity in binding to G4-RNA, with TDP-43 recognizing parallel-stranded G4-DNA/RNAs and FUS binding to all three types of G4-DNA/RNAs.
Review
Biochemistry & Molecular Biology
Clement Barbereau, Nicolas Cubedo, Tangui Maurice, Mireille Rossel
Summary: Tauopathies encompass a diverse range of neurodegenerative diseases, with Alzheimer's disease being the most prominent one. Accumulation of abnormal forms of Tau protein in neural cells leads to its aggregation and neurofibrillary tangles, contributing to cognitive deficits and locomotor problems. Zebrafish transgenic models have been instrumental in studying Tau protein interactions and exploring neuroprotective approaches against Tau-related pathologies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Richard Bedlack, Emma Fixsen, Colin Quinn, Chafic Karam, Alex Sherman, Lyle Ostrow, Orla Hardiman, Terry Heiman-Patterson, Laurie Gutmann, Mark Bromberg, Gregory Carter, Edor Kabashi, Tulio Bertorini, Tahseen Mozaffar, Peter Andersen, Josep Gamez, Mazen Dimachkie, Yunxia Wang, Paul Wicks, James Heywood, Steven Novella, L. P. Rowland, Erik Pioro, Lisa Kinsley, Kathy Mitchell, Jonathan Glass, Sith Sathornsumetee, Jon Baker, Nazem Atassi, Dallas Forshew, John Ravits, Robin Conwit, Carlayne Jackson, Kate Dalton, Katherine Tindall, Ginna Gonzalez, Janice Robertson, Larry Phillips, Michael Benatar, Eric Sorenson, Christen Shoesmith, Steven Nash, Nicholas Maragakis, Dan Moore, James Caress, Kevin Boylan, Carmel Armon, Megan Grosso, Bonnie Gerecke, Jim Wymer, Bjorn Oskarsson, Robert Bowser, Vivian Drory, Jeremy Shefner, Noah Lechtzin, Melanie Leitner, Robert Miller, Todd Levine, James Russell, Khema Sharma, David Saperstein, Leo McClusky, Daniel MacGowan, Jonathan Licht, Ashok Verma, Michael Strong, Catherine Lomen-Hoerth, Rup Tandan, Michael Rivner, Steve Kolb, Meraida Polak, Stacy Rudnicki, Pamela Kittrell, Muddasir Quereshi, George Sachs, Gary Pattee, Michael Weiss, John Kissel, Jonathan Goldstein, Jeffrey Rothstein, Dan Pastula, Gleb Levitsky, Mieko Ogino, Jeffrey Rosenfeld, Efrat Carmi, Christina Fournier, Paul Barkhaus, Brett Morrison, Lome Zinman, Eric Valor, Neta Zach, Ahmad Ghavanini, Yvonne Baker, Kristiana Salmon, Steve Perrin, Rob Goldstein, Fernando Vieira, Merit Cudkowicz, Hiroshi Mitsumoto
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
(2017)
Article
Clinical Neurology
Sabrina Paganoni, Richard Bedlack, Emma Fixsen, Colin Quinn, Chafic Karam, Alex Sherman, Lyle Ostrow, Orla Hardiman, Terry Heiman-Patterson, Laurie Gutmann, Mark Bromberg, Gregory Carter, Edor Kabashi, Tulio Bertorini, Tahseen Mozaffar, Peter Andersen, Josep Gamez, Mazen Dimachkie, Yunxia Wang, Paul Wicks, James Heywood, Steven Novella, L. P. Rowland, Erik Pioro, Lisa Kinsley, Kathy Mitchell, Jonathan Glass, Sith Sathornsumetee, Jon Baker, Nazem Atassi, Dallas Forshew, John Ravits, Robin Conwit, Carlayne Jackson, Kate Dalton, Katherine Tindall, Ginna Gonzalez, Janice Robertson, Larry Phillips, Michael Benatar, Eric Sorenson, Christen Shoesmith, Steven Nash, Nicholas Maragakis, Dan Moore, James Caress, Kevin Boylan, Carmel Armon, Megan Grosso, Bonnie Gerecke, Jim Wymer, Bjorn Oskarsson, Robert Bowser, Vivian Drory, Jeremy Shefner, Noah Lechtzin, Melanie Leitner, Robert Miller, Todd Levine, James Russell, Khema Sharma, David Saperstein, Leo McClusky, Daniel MacGowan, Jonathan Licht, Ashok Verma, Michael Strong, Catherine Lomen-Hoerth, Rup Tandan, Michael Rivner, Steve Kolb, Meraida Polak, Stacy Rudnicki, Pamela Kittrell, Muddasir Quereshi, George Sachs, Gary Pattee, Michael Weiss, John Kissel, Jonathan Goldstein, Jeffrey Rothstein, Dan Pastula, Gleb Levitsky, Mieko Ogino, Jeffrey Rosenfeld, Efrat Carmi, Christina Fournier, Paul Barkhaus, Brett Morrison, Lorne Zinman, Eric Valor, Neta Zach, Ahmad Ghavanini, Yvonne Baker, Kristiana Salmon, Steve Perrin, Rob Goldstein, Fernando Vieira, Merit Cudkowicz, Hiroshi Mitsumoto, Keelie Denson, Ceri Weber
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
(2017)
Article
Clinical Neurology
Richard Bedlack, Colin Quinn, Chafic Karam, Alex Sherman, Lyle Ostrow, Orla Hardiman, Terry Heiman-Patterson, Laurie Gutmann, Mark Bromberg, Gregory Carter, Edor Kabashi, Tulio Bertorini, Tahseen Mozaffar, Peter Andersen, Jeff Dietz, Josep Gamez, Mazen Dimachkie, Yunxia Wang, Paul Wicks, James Heywood, Steven Novella, L. P. Rowland, Erik Pioro, Lisa Kinsley, Kathy Mitchell, Jonathan Glass, Sith Sathornsumetee, Jon Baker, Nazem Atassi, Dallas Forshew, John Ravits, Robin Conwit, Carlayne Jackson, Kate Dalton, Katherine Tindall, Ginna Gonzalez, Janice Robertson, Larry Phillips, Michael Benatar, Eric Sorenson, Christen Shoesmith, Steven Nash, Nicholas Maragakis, Dan Moore, James Caress, Kevin Boylan, Carmel Armon, Megan Grosso, Bonnie Gerecke, Jim Wymer, Bjorn Oskarsson, Robert Bowser, Vivian Drory, Jeremy Shefner, Noah Lechtzin, Melanie Leitner, Robert Miller, Hiroshi Mitsumoto, Todd Levine, James Russell, Khema Sharma, David Saperstein, Leo McClusky, Daniel MacGowan, Jonathan Licht, Ashok Verma, Michael Strong, Catherine Lomen-Hoerth, Rup Tandan, Michael Rivner, Steve Kolb, Meraida Polak, Stacy Rudnicki, Pamela Kittrell, Muddasir Quereshi, George Sachs, Gary Pattee, Michael Weiss, John Kissel, Jonathan Goldstein, Jeffrey Rothstein, Dan Pastula, Gleb Levitsky, Mieko Ogino, Jeffrey Rosenfeld, Efrat Carmi, Merit Cudkowicz, Christina Fournier, Paul Barkhaus, Brett Morrison, Lorne Zinman, Craig Oster, Eric Valor, Neta Zach, Ahmad Ghavanini, Yvonne Baker, Kristiana Salmon
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
(2016)
Article
Clinical Neurology
Veronica Peschansky, Richard Bedlack, Colin Quinn, Chafic Karam, Alex Sherman, Lyle Ostrow, Orla Hardiman, Terry Heiman-Patterson, Laurie Gutmann, Mark Bromberg, Gregory Carter, Edor Kabashi, Tulio Bertorini, Tahseen Mozaffar, Peter Andersen, Josep Gamez, Mazen Dimachkie, Yunxia Wang, Paul Wicks, James Heywood, Steven Novella, L. P. Rowland, Erik Pioro, Lisa Kinsley, Kathy Mitchell, Jonathan Glass, Sith Sathornsumetee, Jon Baker, Nazem Atassi, Dallas Forshew, John Ravits, Robin Conwit, Carlayne Jackson, Kate Dalton, Katherine Tindall, Ginna Gonzalez, Janice Robertson, Larry Phillips, Michael Benatar, Eric Sorenson, Christen Shoesmith, Steven Nash, Nicholas Maragakis, Dan Moore, James Caress, Kevin Boylan, Carmel Armon, Megan Grosso, Bonnie Gerecke, Jim Wymer, Bjorn Oskarsson, Robert Bowser, Vivian Drory, Jeremy Shefner, Noah Lechtzin, Melanie Leitner, Robert Miller, Todd Levine, James Russell, Khema Sharma, David Saperstein, Leo McClusky, Daniel MacGowan, Jonathan Licht, Ashok Verma, Michael Strong, Catherine Lomen-Hoerth, Rup Tandan, Michael Rivner, Steve Kolb, Meraida Polak, Stacy Rudnicki, Pamela Kittrell, Muddasir Quereshi, George Sachs, Gary Pattee, Michael Weiss, John Kissel, Jonathan Goldstein, Jeffrey Rothstein, Dan Pastula, Gleb Levitsky, Mieko Ogino, Jeffrey Rosenfeld, Efrat Carmi, Christina Fournier, Paul Barkhaus, Brett Morrison, Lorne Zinman, Eric Valour, Neta Zach, Ahmad Ghavanini, Yvonne Baker, Kristiana Salmon, Steve Perrin, Rob Goldstein, Fernando Vieira, Merit Cudkowicz, Hiroshi Mitsumoto, Emma Fixen, Ceri Weber, Keeli Hope Denson
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
(2017)
Article
Geriatrics & Gerontology
William Sproviero, Aleksey Shatunov, Daniel Stahl, Maryam Shoai, Wouter van Rheenen, Ashley R. Jones, Safa Al-Sarraj, Peter M. Andersen, Nancy M. Bonini, Francesca L. Conforti, Philip Van Damme, Hussein Daoud, Maria Del Mar Amador, Isabella Fogh, Monica Forzan, Ben Gaastra, Cinzia Gellera, Aaron D. Gitler, John Hardy, Pietro Fratta, Vincenzo La Bella, Isabelle Le Ber, Tim Van Langenhove, Serena Lattante, Yi-Chung Lee, Andrea Malaspina, Vincent Meininger, Stephanie Millecamps, Richard Orrell, Rosa Rademakers, Wim Robberecht, Guy Rouleau, Owen A. Ross, Francois Salachas, Katie Sidle, Bradley N. Smith, Bing-Wen Soong, Gianni Soraru, Giovanni Stevanin, Edor Kabashi, Claire Troakes, Christine van Broeckhoven, Jan H. Veldink, Leonard H. van den Berg, Christopher E. Shaw, John F. Powell, Ammar Al-Chalabi
NEUROBIOLOGY OF AGING
(2017)
Article
Clinical Neurology
Hortense de Calbiac, Adriana Dabacan, Elise Marsan, Herve Tostivint, Gabrielle Devienne, Saeko Ishida, Eric Leguern, Stephanie Baulac, Raul C. Muresan, Edor Kabashi, Sorana Ciura
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2018)
Article
Neurosciences
Alexandra Lissouba, Meijiang Liao, Edor Kabashi, Pierre Drapeau
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2018)
Review
Clinical Neurology
Maria-Letizia Campanari, Annis-Rayan Bourefis, Edor Kabashi
FRONTIERS IN NEUROLOGY
(2019)
Article
Cell Biology
Doris Lou Demy, Maria Letizia Campanari, Raphael Munoz-Ruiz, Heather D. Durham, Benoit J. Gentil, Edor Kabashi
Article
Cell Biology
Maria-Letizia Campanari, Anca Marian, Sorana Ciura, Edor Kabashi
Summary: The study suggests that AChE plays a crucial role in ALS pathology by regulating NMJ function. Knockdown of TDP-43 in zebrafish led to NMJ dysfunction, which was partially rescued by overexpression of human AChE. This finding provides insights into the early defects in NMJs in ALS and highlights AChE as a potential therapeutic target for the disease.
Review
Cell Biology
Jason P. Chua, Hortense De Calbiac, Edor Kabashi, Sami J. Barmada
Summary: Protein homeostasis mechanisms are crucial for maintaining the health of neurons and other cells in the central nervous system, while autophagy plays a key role in preventing and combating pathogenic insults that may lead to neurodegenerative diseases. However, the specific mechanisms underlying the neuroprotective role of autophagy, neuronal resistance to autophagy induction, and the effects of autophagy-impairing mutations on neurons are not fully understood. Additionally, the contribution of non-cell autonomous effects of autophagy dysfunction to ALS pathogenesis remains incompletely defined.
Article
Multidisciplinary Sciences
Maria-Letizia Campanari, Annis-Rayan Bourefis, Valerie Buee-Scherrer, Edor Kabashi
Article
Clinical Neurology
Christel Depienne, Sorana Ciura, Oriane Trouillard, Delphine Bouteiller, Elsa Leitao, Caroline Nava, Boris Keren, Yannick Marie, Justine Guegan, Sylvie Forlani, Alexis Brice, Mathieu Anheim, Yves Agid, Paul Krack, Philippe Damier, Francois Viallet, Jean-Luc Houeto, Franck Durif, Marie Vidailhet, Yulia Worbe, Emmanuel Roze, Edor Kabashi, Andreas Hartmann
TREMOR AND OTHER HYPERKINETIC MOVEMENTS
(2019)
Article
Medicine, Research & Experimental
Shunmoogum A. Patten, Dina Aggad, Jose Martinez, Elsa Tremblay, Janet Petrillo, Gary A. B. Armstrong, Alexandre La Fontaine, Claudia Maios, Meijiang Liao, Sorana Ciura, Xiao-Yan Wen, Victor Rafuse, Justin Ichida, Lorne Zinman, Jean-Pierre Julien, Edor Kabashi, Richard Robitaille, Lawrence Korngut, J. Alexander Parker, Pierre Drapeau