ArticleBMC neuroscience2017
Myelination is delayed during postnatal brain development in the mdx mouse model of Duchenne muscular dystrophy.
Article in BMC neuroscience, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Tissue- and cell-specific whole-transcriptome meta-analysis from brain and retina reveals differential expression of dystrophin complexes and new dystrophin spliced isoforms.Human molecular genetics · 2023Pooled it
- Relationships betweenNeurology · 2019Trial
- Loss of dystrophin reduces CB1 receptor expression and endocannabinoid-dependent synaptic plasticity in the cerebellar cortex.bioRxiv : the preprint server for biology · 2026Article
- Article
- The role of dystrophin isoforms and interactors in the brain.Brain : a journal of neurology · 2025Review
- Cognitive abnormalities in Becker muscular dystrophy: a mysterious link between dystrophin deficiency and executive functions.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Article
- Dmd mdx mice have defective oligodendrogenesis, delayed myelin compaction and persistent hypomyelination.Disease models & mechanisms · 2024Article
- Age-associated sleep spindle characteristics in Duchenne muscular dystrophy.Sleep advances : a journal of the Sleep Research Society · 2024Article
- Whole-Exome Sequencing Identifies Genetic Variants for Severe Adolescent Idiopathic Scoliosis in a Taiwanese Population.Journal of personalized medicine · 2022Article
- Neuroimaging evidence of glymphatic system dysfunction in possible REM sleep behavior disorder and Parkinson's disease.NPJ Parkinson's disease · 2022Article
- Disrupted structural connectome and neurocognitive functions in Duchenne muscular dystrophy: classifying and subtyping based on Dp140 dystrophin isoform.Journal of neurology · 2022Article
- Autism Spectrum Disorder and Duchenne Muscular Dystrophy: A Clinical Case on the Potential Role of the Dystrophin in Autism Neurobiology.Journal of clinical medicine · 2021Article
- Emotional behavior and brain anatomy of the mdx52 mouse model of Duchenne muscular dystrophy.Disease models & mechanisms · 2021Article
- Longitudinal Evaluation of Working Memory in Duchenne Muscular Dystrophy.Journal of clinical medicine · 2020Article
- In Vivo Evaluation of White Matter Abnormalities in Children with Duchenne Muscular Dystrophy Using DTI.AJNR. American journal of neuroradiology · 2020Article
- Comprehensive analysis of the expression profile of circRNAs and their predicted protein-coding ability in the muscle of mdx mice.Functional & integrative genomics · 2020Article
- Regulation of Oligodendrocyte Functions: Targeting Lipid Metabolism and Extracellular Matrix for Myelin Repair.Journal of clinical medicine · 2020Review
- The dystroglycan receptor maintains glioma stem cells in the vascular niche.Acta neuropathologica · 2019Article
- Neurocognitive Impairment in mdx Mice.Molecular neurobiology · 2019Article
- The Role ofAnnals of neurosciences · 2019Article
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3 authors.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundIn Duchenne muscular dystrophy (DMD), the loss of the dystrophin component of the dystrophin-glycoprotein complex (DGC) compromises plasma membrane integrity in skeletal muscle, resulting in extensive muscle degeneration. In addition, many DMD patients exhibit brain deficits in which the cellular etiology remains poorly understood. We recently found that dystroglycan, a receptor component of the DGC that binds intracellularly to dystrophin, regulates the development of oligodendrocytes, the myelinating glial cells of the brain.
resultsWe investigated whether dystrophin contributes to oligodendroglial function and brain myelination. We found that oligodendrocytes express up to three dystrophin isoforms, in conjunction with classic DGC components, which are developmentally regulated during differentiation and in response to extracellular matrix engagement. We found that mdx mice, a model of DMD lacking expression of the largest dystrophin isoform, have delayed myelination and inappropriate oligodendrocyte progenitor proliferation in the cerebral cortex. When we prevented the expression of all oligodendroglial dystrophin isoforms in cultured oligodendrocytes using RNA interference, we found that later stages of oligodendrocyte maturation were significantly delayed, similar to mdx phenotypes in the developing brain.
conclusionsWe find that dystrophin is expressed in oligodendrocytes and influences developmental myelination, which provides new insight into potential cellular contributors to brain dysfunction associated with DMD.
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