Review
Clinical Neurology
Philippe Corcia, Christian Lunetta, Patrick Vourc'h, Pierre-Francois Pradat, Helene Blasco
Summary: This article reviews the progress in the diagnosis, monitoring, and treatment of amyotrophic lateral sclerosis (ALS). Despite the difficulty in diagnosing and the lack of a cure for ALS, there is evidence to suggest that an optimistic view of ALS management in the coming years is now realistic.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Biochemistry & Molecular Biology
Jose R. Monteiro Neto, Gabriela D. Ribeiro, Rayne S. S. Magalhaes, Cristian Follmer, Tiago F. Outeiro, Elis C. A. Eleutherio
Summary: This study found a relationship between the formation of methylglyoxal (MGO) and the degeneration of motor neurons in Amyotrophic Lateral Sclerosis (ALS). The accumulation of MGO led to the aggregation of human SOD1WT (hSOD1WT), decreased activity, and reduced cell viability. Additionally, MGO treatment increased the presence of hSOD1WT in stress granules. These findings suggest that glycation may play a significant role in the pathologies of hSOD1WT and TDP-43 in sporadic ALS.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2023)
Article
Neurosciences
Xiaojiao Xu, Jingjing Zhang, Song Li, Murad Al-Nusaif, Qinming Zhou, Sheng Chen, Weidong Le
Summary: The study found that neuroinflammation in the early pathogenesis of ALS is associated with increased immune-related genes, and BST2 may serve as a potential target for ameliorating microglia-mediated neuroinflammation pathologies in ALS.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Medicine, General & Internal
Wen-Chao Liu, Na Liu, Yan Wang, Chen Huang, Yan-Fang Li, Hao Wang, Xiao-Gang Li, Min Deng
Summary: Research shows that motor neurons (MNs) derived from ALS patient-specific iPSC lines can replicate key aspects of ALS pathogenesis, providing insights into the disease's pathophysiological processes. Incremental mutant expressions of SOD1 in MNs may disrupt cellular function, leading to intracellular calcium disturbances and contributing to the onset of the disease.
CHINESE MEDICAL JOURNAL
(2021)
Article
Neurosciences
Albert J. B. Lee, Tyler E. E. Kittel, Renaid B. B. Kim, Thao-Nguyen Bach, Tian Zhang, Cassie S. S. Mitchell
Summary: The study aimed to determine the most beneficial pathophysiological treatment targets for ALS. The results showed that treatments targeting inflammation were best at delaying disease onset, oxidative stress treatments significantly prolonged survival, and excitability treatments improved overall health status. The best pathophysiological treatment category varied with disease progression and combination treatments targeting multiple categories performed better than monotherapies at end-stage.
FRONTIERS IN NEUROSCIENCE
(2023)
Review
Biochemistry & Molecular Biology
Sophie Layalle, Laetitia They, Sarah Ourghani, Cedric Raoul, Laurent Soustelle
Summary: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by the degeneration of motoneurons. Fruit flies have emerged as a versatile model for studying ALS, providing insights into cellular mechanisms and potential therapeutic targets for future treatments. Research on fruit fly ALS models has revealed novel pathogenic mechanisms and identified disease-modifying genes.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Clinical Neurology
Simon Witzel, Kristina Mayer, Patrick Oeckl
Summary: Amyotrophic lateral sclerosis (ALS) is an incurable and devastating neurodegenerative disease with limited treatment options. However, there is promising progress in potential treatments currently being tested in clinical trials. This review discusses recent advances in ALS biomarker research and applications, as well as future directions and needs.
CURRENT OPINION IN NEUROLOGY
(2022)
Article
Physiology
Antonio Mazzaro, Veronica Vita, Marco Ronfini, Irene Casola, Arianna Klein, Gabriella Dobrowolny, Gianni Soraru, Antonio Musaro, Marco Mongillo, Tania Zaglia
Summary: In this study, the sympathetic innervation of normal and ALS muscles was compared through the structural analysis of the sympathetic network in human and murine tissue samples. The results showed that ALS murine muscles exhibited denervation of sympathetic neurons, which began in the early stage of the disease and worsened with aging. Similar neuronal degeneration was also observed in muscle biopsies from an ALS patient carrying the SOD1(G93A) mutation. This suggests that sympathetic neurons are also compromised in ALS and that dysfunctional muscles in ALS affect their sympathetic innervation.
FRONTIERS IN PHYSIOLOGY
(2023)
Article
Clinical Neurology
Delia Gagliardi, Paolo Ripellino, Megi Meneri, Roberto Del Bo, Sara Antognozzi, Giacomo Pietro Comi, Claudio Gobbi, Antonia Ratti, Nicola Ticozzi, Vincenzo Silani, Dario Ronchi, Stefania Corti
Summary: In this study, the authors provided a clinical and molecular description of a cohort of SOD1-ALS patients, revealing the heterogeneity in clinical and molecular characteristics of SOD1 mutations. The cohort exhibited variable expressivity, atypical presentations, and different modes of inheritance. With the availability of SOD1-directed antisense oligonucleotide for SOD1-ALS patients, prompt screening for SOD1 mutations in ALS patients is recommended.
FRONTIERS IN NEUROLOGY
(2023)
Article
Neurosciences
Rebecca Stevenson, Evgeniia Samokhina, Armaan Mangat, Ilaria Rossetti, Sushmitha S. Purushotham, Chandra S. Malladi, John W. Morley, Yossi Buskila
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive loss of motor neurons. Recent studies have shown that astrocytes, a type of glial cells, also contribute to the onset and progression of ALS. This study investigates the role of astrocytes in maintaining K+ homeostasis in the brain and demonstrates region-specific alterations in K+ clearance rate in an ALS mouse model. The findings suggest that impaired astrocytic function may contribute to the vulnerability of motor neurons in ALS.
Article
Medicine, Research & Experimental
Takayuki Kondo, Ikuyo Inoue, Kazuhiro Umeyama, Masahito Watanabe, Hitomi Matsunari, Ayuko Uchikura, Kazuaki Nakano, Kayoko Tsukita, Keiko Imamura, Hiroshi Nagashima, Haruhisa Inoue
Summary: In this study, a pig model of human mutant SOD1-mediated familial ALS was generated by delivering a genetic construct into porcine zygotes using intra-cytoplasmic sperm injection-mediated gene transfer. The established ALS pig model exhibited initial abnormalities of motor neurons and accumulated misfolded SOD1. This ALS pig model, with a body size similar to that of human beings, could be utilized for cell and gene therapy platforms in preclinical translational research.
LABORATORY INVESTIGATION
(2023)
Article
Pharmacology & Pharmacy
Silvia Scaricamazza, Illari Salvatori, Susanna Amadio, Valentina Nesci, Alessio Torcinaro, Giacomo Giacovazzo, Aniello Primiano, Michela Gloriani, Niccolo Candelise, Luisa Pieroni, Jean-Philippe Loeffler, Frederique Rene, Cyril Quessada, Tesfaye W. Tefera, Hao Wang, Frederik J. Steyn, Shyuan T. Ngo, Gabriella Dobrowolny, Elisa Lepore, Andrea Urbani, Antonio Musaro, Cinzia Volonte, Elisabetta Ferraro, Roberto Coccurello, Cristiana Valle, Alberto Ferri
Summary: The therapeutic potential of the multi-target drug trimetazidine was evaluated in SOD1(G93A) mice. The results showed that trimetazidine delayed disease progression, improved motor function and metabolism, and increased overall survival of the mice.
BRITISH JOURNAL OF PHARMACOLOGY
(2022)
Article
Neurosciences
Isil Keskin, Elaheh Ekhtiari Bidhendi, Matthew Marklund, Peter M. Andersen, Thomas Brannstrom, Stefan L. Marklund, Ulrika Nordstrom
Summary: The deposition of aggregated proteins is a common feature in neurodegenerative disorders. Experimental evidence suggests that disease propagation involves prion-like mechanisms, causing the spreading of specific disease-associated proteins. In mouse models, inoculation of minute amounts of human SOD1 aggregates into the spinal cord or peripheral nerves can induce premature ALS-like disease, but higher doses given through different routes did not lead to disease transmission.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Review
Endocrinology & Metabolism
Nader Akbari Dilmaghani, Bashdar Mahmud Hussen, Saeedeh Nateghinia, Mohammad Taheri, Soudeh Ghafouri-Fard
Summary: ALS, a deadly motor neuron disease, is classified into familial and sporadic types, with genetic risk factors not fully identified in sporadic cases. Studies have shown dysregulation of multiple miRNAs in ALS patients' serum samples and brain tissues, some of which may serve as potential biomarkers for sporadic ALS.
METABOLIC BRAIN DISEASE
(2021)
Article
Neurosciences
Cassandra N. Dennys, Florence Roussel, Rochelle Rodrigo, Xiaojin Zhang, Andrea Sierra Delgado, Annalisa Hartlaub, Asya Saelim-Ector, Will Ray, Sarah Heintzman, Ashley Fox, Stephen J. Kolb, Joseph Beckman, Maria Clara Franco, Kathrin Meyer
Summary: Patient diversity and unknown disease cause pose challenges for drug development and clinical trial design in ALS. Reprogramming patient fibroblasts to neuronal progenitor cells can generate disease relevant cell types for compound testing. CuATSM, currently in clinical trial for ALS, showed a differential effect on neuronal survival in co-culture assays. Elevated mitochondrial respiration was observed in all CuATSM-responders, suggesting a potential metabolic mechanism for CuATSM's therapeutic effects.
Article
Urology & Nephrology
Kimio Sugaya, Saori Nishijima, Katsumi Kadekawa, Katsuhiro Ashitomi, Tomoyuki Ueda, Hideyuki Yamamoto
LUTS-LOWER URINARY TRACT SYMPTOMS
(2017)
Article
Urology & Nephrology
Katsumi Kadekawa, Tsuyoshi Majima, Naoki Kawamorita, Hiroki Okada, Tsuyoshi Yoshizawa, Kenichi Mori, Pradeep Tyagi, Kimio Sugaya, Naoki Yoshimura
NEUROUROLOGY AND URODYNAMICS
(2017)
Article
Urology & Nephrology
Kimio Sugaya, Saori Nishijima, Katsumi Kadekawa, Katsuhiro Ashitomi, Tomoyuki Ueda, Hideyuki Yamamoto, Tsuyoshi Hattori
NEUROUROLOGY AND URODYNAMICS
(2017)
Article
Neurosciences
Katsumi Kadekawa, Tsuyoshi Yoshizawa, Naoki Wada, Takahiro Shimizu, Tsuyoshi Majima, Pradeep Tyagi, William C. de Groat, Kimio Sugaya, Naoki Yoshimura
EXPERIMENTAL NEUROLOGY
(2017)
Article
Medicine, General & Internal
Masako Mukai, Eiichiro Nagata, Atsushi Mizuma, Mitsuhiko Yamano, Keizo Sugaya, Ichizo Nishino, Yu-ichi Goto, Shunya Takizawa
Review
Biology
Kimihiko Sugaya
INTERNATIONAL JOURNAL OF RADIATION BIOLOGY
(2017)
Article
Biochemical Research Methods
Colin Constant, Andrea Bergano, Kiminobu Sugaya, Aristide Dogariu
JOURNAL OF BIOPHOTONICS
(2018)
Review
Medicine, Research & Experimental
Tsuyoshi Hattori, Kimio Sugaya
Article
Environmental Sciences
Kazuki Izawa, Hirokazu Kuwahara, Kaito Sugaya, Nathan Lo, Moriya Ohkuma, Yuichi Hongoh
ENVIRONMENTAL MICROBIOLOGY REPORTS
(2017)
Article
Zoology
Seiki Takatsuki, Risako Miyaoka, Keita Sugaya
ZOOLOGICAL SCIENCE
(2018)
Article
Biology
Frederick Robert Carrick, Sergio F. Azzolino, Melissa Hunfalvay, Guido Pagnacco, Elena Oggero, Ryan C. N. D'Arcy, Mahera Abdulrahman, Kiminobu Sugaya
Summary: The pupillary light reflex (PLR) is modulated by cognitive brain function and can be affected by long-term changes in brain function due to injury. PLR variables such as latency, pupil diameter, constriction velocity, and recovery time show significant differences between subjects with and without concussion history, as well as differences related to gender and symptoms. These differences suggest that PLR metrics can serve as biomarkers for clinical diagnosis and decision making in individuals with concussive injuries.
Article
Biology
Ruben Berrocal Timmons, Kiminobu Sugaya, Lori Deneke Bane
Summary: Injecting cryopreserved umbilical cord tissue (UCT) can alleviate knee pain, improve physical function, and reduce medication use in knee pain subjects.
Article
Biochemistry & Molecular Biology
Manjusha Vaidya, Sandeep Sreerama, Maxine Gonzalez-Vega, Jonhoi Smith, Melvin Field, Kiminobu Sugaya
Summary: Glioblastoma multiforme (GBM) possesses cancer stem cells (CSCs) that contribute to its invasiveness, recurrence, and metastasis. The transcriptional profiles of the cancer cells change when cocultured with neural stem cells (NSCs), resulting in upregulation of stemness and drug resistance genes in GBM and triggering NSCs differentiation. Cell-secreted signaling molecules and extracellular vesicles (EVs) are likely involved in the reciprocal communication and transcription modification between GBM and NSCs.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biology
Frederick Robert Carrick, Luis Sebastian Alexis Valerio, Maxine N. Gonzalez-Vega, David Engel, Kiminobu Sugaya
Summary: Wounds can have a severe impact on the wellbeing of individuals and society, and technology that accelerates wound healing can bring many benefits to injured people. In a study using a ceramic far-field infrared blanket, it was found that wound healing in mice was significantly faster, and mesenchymal stem cells also showed increased wound-healing effects in this environment.
Article
Physiology
Katsumi Kadekawa, Tsuyoshi Majima, Takahiro Shimizu, Naoki Wada, William C. de Groat, Anthony J. Kanai, Momokazu Goto, Mitsuharu Yoshiyama, Kimio Sugaya, Naoki Yoshimura
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY
(2017)