ArticleJournal of neurochemistry2015
Proteomics-level analysis of myelin formation and regeneration in a mouse model for Vanishing White Matter disease.
Article in Journal of neurochemistry, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
20 citing papers in PubMed, 29 citations in OpenAlex.
- The translational landscape of reactive astrocytes reveals the impact of eIF2B-mediated dysregulation in VWM disease.NAR molecular medicine · 2025Article
- Translation dysregulation in neurodegenerative diseases: a focus on ALS.Molecular neurodegeneration · 2023Review
- EIF2B2 gene mutation causing early onset vanishing white matter disease: a case report.Italian journal of pediatrics · 2022Article
- Regulation and function of elF2B in neurological and metabolic disorders.Bioscience reports · 2022Review
- Article
- Proteomics of Multiple Sclerosis: Inherent Issues in Defining the Pathoetiology and Identifying (Early) Biomarkers.International journal of molecular sciences · 2021Review
- Comparative Proteome Research in a Zebrafish Model for Vanishing White Matter Disease.International journal of molecular sciences · 2021Article
- Changes in the Oligodendrocyte Progenitor Cell Proteome with Ageing.Molecular & cellular proteomics : MCP · 2020Article
- Article
- Revisiting the Pathoetiology of Multiple Sclerosis: Has the Tail Been Wagging the Mouse?Frontiers in immunology · 2020Review
- Article
- eIF2B Mutations Cause Mitochondrial Malfunction in Oligodendrocytes.Neuromolecular medicine · 2019Article
- Modeling vanishing white matter disease with patient-derived induced pluripotent stem cells reveals astrocytic dysfunction.CNS neuroscience & therapeutics · 2019Article
- Article
- Drug Screening Identifies Sigma-1-Receptor as a Target for the Therapy of VWM Leukodystrophy.Frontiers in molecular neuroscience · 2018Article
- Mitochondrial malfunction in vanishing white matter disease: a disease of the cytosolic translation machinery.Neural regeneration research · 2017Article
- Partial Immunoblotting of 2D-Gels: A Novel Method to Identify Post-Translationally Modified Proteins Exemplified for the Myelin Acetylome.Proteomes · 2017Article
- Evaluation of the Acquisition of the Aerobic Metabolic Capacity by Myelin, during its Development.Molecular neurobiology · 2016Article
- Glial Contributions to Neural Function and Disease.Molecular & cellular proteomics : MCP · 2016Review
- Role of myelin sheath energy metabolism in neurodegenerative diseases.Neural regeneration research · 2015Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
Vanishing white matter (VWM) is a recessive neurodegenerative disease caused by mutations in translation initiation factor eIF2B and leading to progressive brain myelin deterioration, secondary axonal damage, and death in early adolescence. Eif2b5(R132H/R132H) mice exhibit delayed developmental myelination, mild early neurodegeneration and a robust remyelination defect in response to cuprizone-induced demyelination. In the current study we used Eif2b5(R132H/R132H) mice for mass-spectrometry analyses, to follow the changes in brain protein abundance in normal- versus cuprizone-diet fed mice during the remyelination recovery phase. Analysis of proteome profiles suggested that dysregulation of mitochondrial functions, altered proteasomal activity and impaired balance between protein synthesis and degradation play a role in VWM pathology. Consistent with these findings, we detected elevated levels of reactive oxygen species in mutant-derived primary fibroblasts and reduced 20S proteasome activity in mutant brain homogenates. These observations highlight the importance of tight translational control to precise coordination of processes involved in myelin formation and regeneration and point at cellular functions that may contribute to VWM pathology. Eif2b5(R132H/R132H) mouse model for vanishing white matter (VWM) disease was used for mass spectrometry of brain proteins at two time points under normal conditions and along recovery from cuprizone-induced demyelination. Comparisons of proteome profiles revealed the importance of mitochondrial function and tight coordination between protein synthesis and degradation to myelination formation and regeneration, pointing at cellular functions that contribute to VWM pathology.
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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.