ReviewNeurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics2011
The myelin mutants as models to study myelin repair in the leukodystrophies.
Review in Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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25 citing papers in PubMed, 43 citations in OpenAlex.
- Integrated stress response inhibition prolongs the lifespan of a Pelizaeus-Merzbacher disease mouse model by increasing oligodendrocyte survival.Nature communications · 2025Article
- The Effect of Tumor Necrosis Factor-α and Interleu-Kin-1β on the Restorative Properties of Human Oligodendrocyte Precursor Cells In Vitro.Bioengineering (Basel, Switzerland) · 2025Article
- QSI and DTI of Inherited White Matter Disorders in Rat Spinal Cord: Early Detection and Comparison with Quantitative Electron Microscopy Findings.Diagnostics (Basel, Switzerland) · 2025Article
- Hypomyelination, hypodontia and craniofacial abnormalities in a Polr3b mouse model of leukodystrophy.Brain : a journal of neurology · 2023Article
- Enhancers of Human and Rodent Oligodendrocyte Formation Predominantly Induce Cholesterol Precursor Accumulation.ACS chemical biology · 2022Article
- Mutation of Proteolipid Protein 1 Gene: From Severe Hypomyelinating Leukodystrophy to Inherited Spastic Paraplegia.Biomedicines · 2022Review
- Inhomogeneous magnetization transfer MRI of white matter structures in the hypomyelinated shiverer mouse brain.Magnetic resonance in medicine · 2022Article
- Baicalin Promotes CNS Remyelination via PPARγ Signal Pathway.Neurology(R) neuroimmunology & neuroinflammation · 2022Article
- The multicellular interplay of microglia in health and disease: lessons from leukodystrophy.Disease models & mechanisms · 2021Review
- POLR3-Related Leukodystrophy: Exploring Potential Therapeutic Approaches.Frontiers in cellular neuroscience · 2020Article
- Defining Changes in the Spatial Distribution and Composition of Brain Lipids in the Shiverer and Cuprizone Mouse Models of Myelin Disease.The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society · 2019Article
- Chemical Screening Identifies Enhancers of Mutant Oligodendrocyte Survival and Unmasks a Distinct Pathological Phase in Pelizaeus-Merzbacher Disease.Stem cell reports · 2018Article
- Clemastine effects in rat models of a myelination disorder.Pediatric research · 2018Article
- Activin receptors regulate the oligodendrocyte lineage in health and disease.Acta neuropathologica · 2018Article
- Article
- Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease.Scientific reports · 2016Article
- Targeting human oligodendrocyte progenitors for myelin repair.Experimental neurology · 2016Review
- Inherited and acquired disorders of myelin: The underlying myelin pathology.Experimental neurology · 2016Review
- Modeling the natural history of Pelizaeus-Merzbacher disease.Neurobiology of disease · 2015Article
- Article
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The leukodystrophies are rare and serious genetic disorders of the central nervous system that primarily affect children who frequently die early in life or have significantly delayed motor and mental milestones that result in long-term disability. Although with some of these disorders, early intervention with bone marrow or cord blood transplantation has been proven useful, it has not yet been determined that such therapies promote myelin repair of the central nervous system. Research on experimental therapies aimed at myelin repair is aided by the ability to test cell replacement strategies in genetic models in which the mutations and neuropathology match the human disorder. Thus, models exist of Pelizaeus-Merzbacher disease and the lysosomal storage disorder, Krabbe disease, which reflect the clinical and pathological course of the human disorders. Collectively, animals with mutations in myelin genes are called the myelin mutants, and they include rodent models such as the shiverer mouse that have been extensively used to study myelination by exogenous cell transplantation. These studies have encompassed many permutations of the age of the recipient, type of transplanted cell, site of engraftment, and so forth, and they offer hope that the scaling up of myelin produced by transplanted cells will have clinical significance in treating patients. Here we review these models and discuss their relative importance and use in such translational approaches. We discuss how grafts are identified and functional outcomes are measured. Finally, we briefly discuss the cells that have been successfully transplanted, which may be used in future clinical trials.
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