Fluvoxamine Exerts Sigma-1R to Rescue Autophagy via Pom121-Mediated Nucleocytoplasmic Transport of TFEB
By Chun-Yu Lin, Hsiang-En Wu, Eddie Feng-Ju Weng, Hsuan-Cheng Wu, Tsung-Ping Su, and Shao-Ming Wang Excerpt from the article published in Molecular Neurobioligy (2024). 05 January 2024. DOI: https://doi.org/10.1007/s12035-023-03885-9 Editor’s Highlights Abstract Expansion of the GGGGCC-RNA repeat is a known cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), which currently have no cure. Recent […]
Continue ReadingMitochondria-associated membrane collapse impairs TBK1-mediated proteostatic stress response in ALS
By Seiji Watanabe, Yuri Murata, Yasuyoshi Oka, and Koji Yamanaka Excerpt from the article published in Proceedings of the National Academy of Sciences, November 15, 2023, 120 (47) e2315347120, DOI: https://doi.org/10.1073/pnas.2315347120 Editor’s Highlights Significance Recent studies, including ours, have suggested that mitochondria-associated membrane (MAM) disruption is a common pathogenic mechanism in amyotrophic lateral sclerosis (ALS). In the […]
Continue ReadingStructure-Based Modeling of Sigma 1 Receptor Interactions with Ligands and Cholesterol and Implications for Its Biological Function
By Meewhi Kim and Ilya Bezprozvanny Excerpt from the article published in International Journal of Molecular Sciences 24, no. 16: 12980. 19 August 2023, DOI: https://doi.org/10.3390/ijms241612980 Abstract The sigma 1 receptor (S1R) is a 223-amino-acid-long transmembrane endoplasmic reticulum (ER) protein. The S1R plays an important role in neuronal health and it is an established therapeutic target […]
Continue ReadingPotential roles of the endoplasmic reticulum stress pathway in amyotrophic lateral sclerosis
By Yu-Mi Jeon, Younghwi Kwon, Shinrye Lee, and Hyung-Jun Kim Excerpt from the article published in Frontiers in Aging Neuroscience, 15 February 2023. DOI: https://doi.org/10.3389/fnagi.2023.1047897 Editor’s Highlights Abstract The endoplasmic reticulum (ER) is a major organelle involved in protein quality control and cellular homeostasis. ER stress results from structural and functional dysfunction of the organelle, along […]
Continue ReadingSigma-1 receptor maintains ATAD3A as a monomer to inhibit mitochondrial fragmentation at the mitochondria-associated membrane in amyotrophic lateral sclerosis
By Seiji Watanabe, Mai Horiuchi, Yuri Murata, Okiru Komine, Noe Kawade, Akira Sobue, and Koji Yamanaka Excerpt from the article published in Neurobiology of Disease, 2 February 2023, 106031, ISSN 0969-9961, DOI: https://doi.org/10.1016/j.nbd.2023.106031 Editor’s Highlights Autors’ Highlights Abstract Organelle contact sites are multifunctional platforms for maintaining cellular homeostasis. Alternations of the mitochondria-associated membranes (MAM), one […]
Continue ReadingChaperone-Dependent Mechanisms as a Pharmacological Target for Neuroprotection
By Mikhail V. Voronin, Elena V. Abramova, Ekaterina R. Verbovaya, Yulia V. Vakhitova, and Sergei B. Seredenin Excerpt from the article published in International Journal of Molecular Sciences 24, no. 1: 823. 3 January 2023, DOI: https://doi.org/10.3390/ijms24010823 Editor’s Highlights Abstract Modern pharmacotherapy of neurodegenerative diseases is predominantly symptomatic and does not allow vicious circles causing disease […]
Continue ReadingAmyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation
By Richard J. Mead, Ning Shan, H. Joseph Reiser, Fiona Marshall, and Pamela J. Shaw Excerpt from the article published in Nature Reviews Drug Discovery, 21 December 2022. DOI: https://doi.org/10.1038/s41573-022-00612-2 Abstract Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of motor neurons. As with all major neurodegenerative disorders, development of disease-modifying therapies […]
Continue ReadingSigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling
By Christelle Lasbleiz, Amandine Peyrel, Pauline Tarot, Jérôme Sarniguet, Lucie Crouzier, Nicolas Cubedo, Benjamin Delprat, Mireille Rossel, Tangui Maurice, Jean-Charles Liévens Excerpt from the article published in Redox Biology, 17 November 2022, 102542, ISSN 2213-2317,DOI: https://doi.org/10.1016/j.redox.2022.102542. Editor’s Highlights Autors’ Highlights Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting upper and lower motor neurons. […]
Continue ReadingThe Impact of Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis
By Jiantao Zhao, Xuemei Wang, Zijun Huo, Yanchun Chen, Jinmeng Liu, Zhenhan Zhao, Fandi Meng, Qi Su, Weiwei Bao, Lingyun Zhang, Shuang Wen, Xin Wang, Huancai Liu, and Shuanhu Zhou Excerpt from the article published in Cells 11, no. 13: 2049. Published: 28 June 2022. DOI: https://doi.org/10.3390/cells11132049 Editor’s Highlights Amyotrophic lateral sclerosis (ALS) is a rapidly […]
Continue ReadingEndoplasmic Reticulum and/or Mitochondrial-Dependent Neuronal Degeneration in ALS
By Jennifer S. Sun Article published in NeurologyLive, June 2022, Volume 5, Issue 3 https://www.neurologylive.com/view/endoplasmic-reticulum-mitochondrial-dependent-neuronal-degeneration-als Editor’s Highlights The MAM-associated chaperone protein sigma 1 receptor (Sigma1R) is also involved in lipid export and calcium signaling through IP3R regulation. Agonists of Sigma1R have also been successful in protecting motor neurons and muscle activity in presymptomatic ALS mutSOD1 mice and in […]
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