ArticleFrontiers in molecular neuroscience2018
Drug Screening Identifies Sigma-1-Receptor as a Target for the Therapy of VWM Leukodystrophy.
Article in Frontiers in molecular neuroscience, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 24 citations in OpenAlex.
- Sonic Hedgehog signaling in oligodendrogenesis, myelination, demyelinating diseases, and remyelination.Neural regeneration research · 2026Article
- Pridopidine, a Potent and Selective Therapeutic Sigma-1 Receptor (S1R) Agonist for Treating Neurodegenerative Diseases.Pharmaceuticals (Basel, Switzerland) · 2025Review
- Pharmacological and Pathological Implications of Sigma-1 Receptor in Neurodegenerative Diseases.Biomedicines · 2025Review
- The Effect of Donepezil Hydrochloride in the Twitcher Mouse Model of Krabbe Disease.Molecular neurobiology · 2024Article
- Pridopidine subtly ameliorates motor skills in a mouse model for vanishing white matter.Life science alliance · 2024Article
- Edaravone and mitochondrial transfer as potential therapeutics for vanishing white matter disease astrocyte dysfunction.CNS neuroscience & therapeutics · 2023Article
- Therapy Trial Design in Vanishing White Matter: An Expert Consortium Opinion.Neurology. Genetics · 2022Review
- Novel High Affinity Sigma-1 Receptor Ligands from Minimal Ensemble Docking-Based Virtual Screening.International journal of molecular sciences · 2021Article
- Sigma receptor knockdown augments dysfunction and apoptosis of beta cells induced by palmitate.Experimental biology and medicine (Maywood, N.J.) · 2021Article
- Comparative Proteome Research in a Zebrafish Model for Vanishing White Matter Disease.International journal of molecular sciences · 2021Article
- Primary Cilia in Glial Cells: An Oasis in the Journey to Overcoming Neurodegenerative Diseases.Frontiers in neuroscience · 2021Review
- Vanishing white matter disease expression of truncated EIF2B5 activates induced stress response.eLife · 2020Article
- Glutamate Carrier Involvement in Mitochondrial Dysfunctioning in the Brain White Matter.Frontiers in molecular biosciences · 2020Article
- Astrocyte Subtype Vulnerability in Stem Cell Models of Vanishing White Matter.Annals of neurology · 2019Article
- The Sigma-1 Receptor in Cellular Stress Signaling.Frontiers in neuroscience · 2019Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Vanishing white matter (VWM) disease is an autosomal genetic leukodystrophy caused by mutations in subunits of eukaryotic translation initiation factor 2B (eIF2B). The clinical symptoms exhibit progressive loss of white matter in both hemispheres of the brain, accompanied by motor functions deterioration, neurological deficits, and early death. To date there is no treatment for VWM disease. The aim of this work was to expedite rational development of a therapeutic opportunity. Our approach was to design a computer-aided strategy for an efficient and reliable screening of drug-like molecules; and to use primary cultures of fibroblasts isolated from the Eif2b5
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