ReviewBrain pathology (Zurich, Switzerland)2018
Genetic defects disrupting glial ion and water homeostasis in the brain.
Review in Brain pathology (Zurich, Switzerland), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 53 citations in OpenAlex.
- Written in the Stars: Astrocyte Biology From Evolution to Disease.Acta physiologica (Oxford, England) · 2026Review
- Curing the brain: in search for new astrocyte-specific therapies.Experimental & molecular medicine · 2026Review
- Altered Mechanical Properties of Astrocytes Lacking MLC1: Implications for the Leukodystrophy MLC.Glia · 2026Article
- Aetokthonotoxin, the Causative Agent of Vacuolar Myelinopathy, Uncouples Oxidative Phosphorylation due to Protonophore Activity.Chemical research in toxicology · 2025Article
- Article
- Brain MRI changes in children and young adults with B-cell acute lymphoblastic leukemia following chimeric antigen receptor T-cell therapy.European radiology · 2025Article
- Multiple sclerosis: an immune attack on astrocyte-mediated ion and water homeostasis.Nature reviews. Neurology · 2025Review
- Neuroglial Pathophysiology of Leukodystrophies.Advances in neurobiology · 2025Review
- Higher sodium in older individuals or after stroke/reperfusion, but not in migraine or Alzheimer's disease - a study in different preclinical models.Scientific reports · 2024Article
- Astrocytes in functional recovery following central nervous system injuries.The Journal of physiology · 2024Review
- When brain size matters: novel insights into brain volume control.Brain : a journal of neurology · 2023Article
- Aquaporin-4 and GPRC5B: old and new players in controlling brain oedema.Brain : a journal of neurology · 2023Article
- The CaMKII/MLC1 Axis Confers CaCells · 2022Article
- Heterogeneity of white matter astrocytes in the human brain.Acta neuropathologica · 2022Review
- Mechanisms of cytokine release syndrome and neurotoxicity of CAR T-cell therapy and associated prevention and management strategies.Journal of experimental & clinical cancer research : CR · 2021Review
- SOX9-induced Generation of Functional Astrocytes Supporting Neuronal Maturation in an All-human System.Stem cell reviews and reports · 2021Article
- Human iPSC-Derived Glia as a Tool for Neuropsychiatric Research and Drug Development.International journal of molecular sciences · 2021Review
- Pathology of the neurovascular unit in leukodystrophies.Acta neuropathologica communications · 2021Article
- The Role of Astrocytes in the Modulation ofKInternational journal of molecular sciences · 2020Review
- SIK3 suppresses neuronal hyperexcitability by regulating the glial capacity to buffer KThe Journal of cell biology · 2019Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Electrical activity of neurons in the brain, caused by the movement of ions between intracellular and extracellular compartments, is the basis of all our thoughts and actions. Maintaining the correct ionic concentration gradients is therefore crucial for brain functioning. Ion fluxes are accompanied by the displacement of osmotically obliged water. Since even minor brain swelling leads to severe brain damage and even death, brain ion and water movement has to be tightly regulated. Glial cells, in particular astrocytes, play a key role in ion and water homeostasis. They are endowed with specific channels, pumps and carriers to regulate ion and water flow. Glial cells form a large panglial syncytium to aid the uptake and dispersal of ions and water, and make extensive contacts with brain fluid barriers for disposal of excess ions and water. Genetic defects in glial proteins involved in ion and water homeostasis disrupt brain functioning, thereby leading to neurological diseases. Since white matter edema is often a hallmark disease feature, many of these diseases are characterized as leukodystrophies. In this review we summarize our current understanding of inherited glial diseases characterized by disturbed brain ion and water homeostasis by integrating findings from MRI, genetics, neuropathology and animal models for disease. We discuss how mutations in different glial proteins lead to disease, and highlight the similarities and differences between these diseases. To come to effective therapies for this group of diseases, a better mechanistic understanding of how glial cells shape ion and water movement in the brain is crucial.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.