Review
Clinical Neurology
YiMing Yuan, Yan Wang, ShanHong Wu, Ming Yue Zhao
Summary: Traumatic peripheral nerve injury is a common and significant clinical problem that can result in functional impairment and permanent disability. Despite advancements in diagnostic procedures and surgical techniques, recovery after peripheral nerve repair is often unsatisfactory. Understanding the role of myelin and myelination in the peripheral nervous system is crucial for promoting nerve regeneration.
FRONTIERS IN NEUROLOGY
(2022)
Article
Immunology
Guillaume Taieb, Alexandre Jentzer, Elisa Vegezzi, Cinta Lleixa, Isabel Illa, Luis Querol, Jerome J. Devaux
Summary: Autoimmune nodopathies (AN) are a subset of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients who have no or poor response to intravenous immunoglobulins. The biomarkers of AN are autoantibodies, mainly IgG4, that target the paranodal region of nerve fibers. This study found that most antibodies in AN patients are bispecific and that functionally monovalent antibodies can alter the paranodal region, but their presence does not correlate with disease severity.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Raquel Gomez-Sintes, Esperanza Arias
Summary: Chaperone-mediated autophagy (CMA) is a proteolytic process that selectively degrades intracellular proteins inside lysosomes, participating in the modulation of specific regulatory proteins. Impairment of CMA function may contribute to the pathogenesis of neurodegenerative diseases and cancer, making it a potential therapeutic target for these conditions. Recent evidence has revealed the important and complex role of CMA in cancer biology, with implications for tumor development and the response to cancer therapeutics.
MOLECULAR ASPECTS OF MEDICINE
(2021)
Article
Multidisciplinary Sciences
Jennifer Cable, Eilika Weber-Ban, Tim Clausen, Kylie J. Walters, Michal Sharon, Daniel J. Finley, Yangnan Gu, John Hanna, Yue Feng, Sascha Martens, Anne Simonsen, Malene Hansen, Hong Zhang, Jonathan M. Goodwin, Alessio Reggio, Chunmei Chang, Liang Ge, Brenda A. Schulman, Raymond J. Deshaies, Ivan Dikic, J. Wade Harper, Ingrid E. Wertz, Nicolas H. Thoma, Mikolaj Slabicki, Judith Frydman, Ursula Jakob, Della C. David, Eric J. Bennett, Carolyn R. Bertozzi, Richa Sardana, Vinay V. Eapen, Serena Carra
Summary: Targeted protein degradation is essential for cellular function and development. This process involves tightly regulated protein degradation pathways to eliminate misfolded and aggregated proteins, adjust protein levels during cellular differentiation, and selectively eliminate target proteins. Understanding these pathways can provide insights into disease pathology and the development of novel therapeutics.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2022)
Article
Neurosciences
Mariapaola Sidoli, Chelsey B. Reed, Cristina Scapin, Pablo Paez, Douglas R. Cavener, Randal J. Kaufman, Maurizio D'Antonio, M. Laura Feltri, Lawrence Wrabetz
Summary: Despite the interaction between Perk and calcineurin in P0S63del nerves, Perk deletion paradoxically improved S63del myelin defects. Additionally, genetic manipulation of calcineurin subunits showed context-dependent protective or toxic effects in S63del, suggesting high sensitivity of Schwann cells to alterations in calcineurin activity.
JOURNAL OF NEUROSCIENCE
(2021)
Review
Biochemistry & Molecular Biology
Lion Borgert, Swadha Mishra, Fabian den Brave
Summary: The nucleus plays an emerging role in cellular quality control by degrading or sequestering aberrant proteins, thus protecting the cellular environment.
COMPUTATIONAL AND STRUCTURAL BIOTECHNOLOGY JOURNAL
(2022)
Article
Multidisciplinary Sciences
Hubert Wyszkowski, Anna Janta, Wiktoria Sztangierska, Igor Obuchowski, Tomasz Chamera, Agnieszka Klosowska, Krzysztof Liberek
Summary: The study investigates the functional differences between two classes of JDPs in yeast during the process of protein disaggregation, highlighting the superior aggregate binding ability of Ydj1 and the recruitment of more Ssa1 molecules to substrates by Sis1, which depends on its specific binding mechanism with Hsp70. These findings suggest that the subspecialization of JDPs during protein reactivation improves the robustness and efficiency of the disaggregation machinery.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Clinical Neurology
Alan Pestronk, Robert E. Schmidt, Robert Bucelli, Julia Sim
Summary: This study examined the protein components of Schwann cells and myelin in normal and diseased human peripheral nerves, and found that the molecular composition of the central nervous system varies with age, axon size, and nerve pathology.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Tomohiro Nakamura, Chang-ki Oh, Xu Zhang, Stuart A. Lipton
Summary: Neurodegenerative disorders such as Alzheimer's and Parkinson's diseases are characterized by progressive degeneration of synapses and neurons, often attributed to accumulated misfolded/aggregated proteins. Excessive reactive oxygen and nitrogen species in the brain may contribute to protein misfolding, potentially exacerbated by genetic mutations and environmental factors. Understanding the role of reactive nitrogen species in post-translational modifications of proteins could lead to therapeutic interventions for neurodegenerative diseases.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Review
Pharmacology & Pharmacy
Hiroko Baba
Summary: Myelin is a multilamellar membrane structure formed by cells in the central and peripheral nervous system. It serves as an insulator and interacts with axons to regulate the nervous system. Recent studies have shown that myelin dynamically changes axons and divides them into different functional domains. The discovery of autoantibodies in patients with peripheral neuropathy has sparked interest in myelin research.
YAKUGAKU ZASSHI-JOURNAL OF THE PHARMACEUTICAL SOCIETY OF JAPAN
(2022)
Article
Biochemistry & Molecular Biology
Lena Kraemer, Niko Dalheimer, Markus Raeschle, Zuzana Storchova, Jan Pielage, Felix Boos, Johannes M. Herrmann
Summary: The cytosol can transiently store mitochondrial precursor proteins in dedicated deposits called MitoStores, which suppress the toxic potential of accumulating precursor proteins and are controlled by Hsp42 and Hsp104.
Review
Chemistry, Multidisciplinary
Alexander Kister, Ilya Kister
Summary: Myelin is a modified cell membrane that surrounds the axon, providing electrical insulation and trophic support. It differs from other cell membranes in terms of composition, with unique lipid components and specific proteins such as myelin basic protein, proteolipid protein, and myelin protein zero. Understanding the functions of myelin is important for studying nerve impulse propagation and neurologic diseases like multiple sclerosis. Future research should focus on exploring the history of discovery in this field and addressing remaining questions.
FRONTIERS IN CHEMISTRY
(2023)
Review
Cell Biology
Xiao-Qiong Chen, Tao Shen, Shao-Jun Fang, Xiao-Min Sun, Guo-Yu Li, Yun-Feng Li
Summary: Aging is a significant risk factor for cancer, as both aging and cancer are characterized by dysfunction in protein homeostasis. Understanding the mechanisms of proteostasis regulation and its relationship with aging and age-related diseases, including cancer, can contribute to improving the health and quality of life for older individuals. Furthermore, maintaining proteostasis offers potential clinical applications in delaying the aging process and promoting long-term health.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Review
Clinical Neurology
Alessandra Bolino
Summary: Myelin, formed by the plasma membrane of glial cells, insulates axons in the nervous system of vertebrates and plays a crucial role in speeding up electric signal transmission and influencing axonal metabolism and neural circuit plasticity. By studying Schwann cells and their myelination process, as well as human disorders related to myelination, researchers have significantly advanced our understanding of myelin biology.
Article
Biochemistry & Molecular Biology
Young Rae Jo, Yuna Oh, Young Hee Kim, Yoon Kyung Shin, Hye Ran Kim, Hana Go, Jaekyoon Shin, Hye Ji Park, Hyongjong Koh, Jong Kuk Kim, Jung Eun Shin, Kyung Eun Lee, Hwan Tae Park
Summary: DSCs utilize autophagy-related genes to form secretory phagophores and tubular phagophores, enabling the exocytosis of large myelin chambers. This autophagy-mediated myelin clearance mechanism is a novel response of DSCs to nerve damage.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2023)