In cancer and cardiovascular disease, endothelial cells can transform into mesenchymal cells (EndoMT), affecting disease onset and progression. In this issue of Neuron, Sun et al. (2022) demonstrate how EndoMT triggers breakdown of the blood-CNS barrier in the pathogenesis of multiple sclerosis.
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| http://dx.doi.org/10.1016/j.neuron.2022.09.017 | DOI Listing |
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Oxidative/Nitrosative Stress, Apoptosis, and Redox Signaling: Key Players in Neurodegenerative Diseases.
J Biochem Mol Toxicol
January 2025
Department of Medical Biochemistry, Faculty of Medicine, Kahramanmaraş Sütçü İmam University, Kahramanmaraş, Turkey.
Neurodegenerative diseases are significant health concerns that have a profound impact on the quality and duration of life for millions of individuals. These diseases are characterized by pathological changes in various brain regions, specific genetic mutations associated with the disease, deposits of abnormal proteins, and the degeneration of neurological cells. As neurodegenerative disorders vary in their epidemiological characteristics and vulnerability of neurons, treatment of these diseases is usually aimed at slowing disease progression.
View Article and Find Full Text PDFArtificial enforcement of the unfolded protein response (UPR) reduces disease features in multiple preclinical models of ALS/FTD.
Mol Ther
January 2025
Program of Cellular and Molecular Biology, Biomedical Sciences Institute (ICBM), Universidad de Chile, Santiago, Chile; Biomedical Neuroscience, Faculty of Medicine, Universidad de Chile, Santiago, Chile; FONDAP Center for Geroscience, Brain Health and Metabolism, Santiago, Chile; Buck Institute for Research on Aging, Novato, CA, USA. Electronic address:
Amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD) are part of a spectrum of diseases that share several causative genes, resulting in a combinatory of motor and cognitive symptoms and abnormal protein aggregation. Multiple unbiased studies have revealed that proteostasis impairment at the level of the endoplasmic reticulum (ER) is a transversal pathogenic feature of ALS/FTD. The transcription factor XBP1s is a master regulator of the unfolded protein response (UPR), the main adaptive pathway to cope with ER stress.
View Article and Find Full Text PDFLoss of Insight in Syndromes Associated with Frontotemporal Lobar Degeneration: Clinical and Imaging Features.
Am J Geriatr Psychiatry
December 2024
Department of Clinical and Experimental Sciences (DA, BB), University of Brescia, Brescia, Italy; Molecular Markers Laboratory (BB), IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy. Electronic address:
Objectives: The present study aims to assess the prevalence, associated clinical symptoms, longitudinal changes, and imaging correlates of Loss of Insight (LOI), which is still unexplored in syndromes associated with Frontotemporal Lobar Degeneration (FTLD).
Design: Retrospective longitudinal cohort study, from Oct 2009 to Feb 2023.
Setting: Tertiary Frontotemporal Dementia research clinic.
The role of injection timing in interferon beta-induced flu-like syndrome in multiple sclerosis: Potential confounders and future directions.
J Clin Neurosci
January 2025
Department of Neurology, School of Medicine, Kyungpook National University, Kyungpook National University Chilgok Hospital, Daegu, Republic of Korea. Electronic address:
C5 complement inhibition versus FcRn modulation in generalised myasthenia gravis.
J Neurol Neurosurg Psychiatry
January 2025
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
Background: Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular junctions, leading to fluctuating muscle weakness. While many patients respond well to standard immunosuppression, a substantial subgroup faces ongoing disease activity. Emerging treatments such as complement factor C5 inhibition (C5IT) and neonatal Fc receptor (FcRn) antagonism hold promise for these patients.
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