ArticleNeurobiology of disease2015
Modeling the natural history of Pelizaeus-Merzbacher disease.
Article in Neurobiology of disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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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
10 citing papers in PubMed, 18 citations in OpenAlex.
- A canineProceedings of the National Academy of Sciences of the United States of America · 2026Article
- Longitudinal natural history studies based on real-world data in rare diseases: Opportunity and a novel approach.Molecular genetics and metabolism · 2024Review
- Pelizaeus-Merzbacher disease: on the cusp of myelin medicine.Trends in molecular medicine · 2024Review
- A novel non-human primate model of Pelizaeus-Merzbacher disease.Neurobiology of disease · 2021Article
- A hypomyelinating leukodystrophy in German Shepherd dogs.Journal of veterinary internal medicine · 2021Article
- Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease.Acta neuropathologica · 2019Article
- Clemastine effects in rat models of a myelination disorder.Pediatric research · 2018Article
- Thin myelin sheaths as the hallmark of remyelination persist over time and preserve axon function.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- Inherited and acquired disorders of myelin: The underlying myelin pathology.Experimental neurology · 2016Review
- Modeling the Chronic Loss of Optic Nerve Axons and the Effects on the Retinal Nerve Fiber Layer Structure in Primary Disorder of Myelin.Investigative ophthalmology & visual science · 2016Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 2 countries.
Funding
Abstract
Major gaps in our understanding of the leukodystrophies result from their rarity and the lack of tissue for the interdisciplinary studies required to extend our knowledge of the pathophysiology of the diseases. This study details the natural evolution of changes in the CNS of the shaking pup (shp), a model of the classical form of the X-linked disorder Pelizaeus-Merzbacher disease, in particular in glia, myelin, and axons, which is likely representative of what occurs over time in the human disease. The mutation in the proteolipid protein gene, PLP1, leads to a delay in differentiation, increased cell death, and a marked distension of the rough endoplasmic reticulum in oligodendrocytes. However, over time, more oligodendrocytes differentiate and survive in the spinal cord leading to an almost total recovery of myelination, In contrast, the brain remains persistently hypomyelinated. These data suggest that shp oligodendrocytes may be more functional than previously realized and that their early recruitment could have therapeutic value.
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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.