Publications by authors named "Paul Magneron"
Brain dysfunction in myotonic dystrophy type 1 (DM1), the prototype of toxic RNA disorders, has been mainly attributed to neuronal RNA misprocessing, while little attention has been given to non-neuronal brain cells. Here, using a transgenic mouse model of DM1 that expresses mutant RNA in various brain cell types (neurons, astroglia, and oligodendroglia), we demonstrate that astrocytes exhibit impaired ramification and polarization in vivo and defects in adhesion, spreading, and migration. RNA-dependent toxicity and phenotypes are also found in human transfected glial cells.
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- - Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder caused by a mutation leading to toxic RNA that disrupts gene processing in various tissues, especially affecting glial cells in the central nervous system (CNS).
- - Research using transcriptomics on DM1 model mice showed significant expression and splicing changes primarily in glial cells, with oligodendrocytes exhibiting the most alterations, indicating issues in cell differentiation.
- - Gene ontology analyses confirmed that these changes in glial cells are linked to critical differentiation processes, and further studies combined with protein analysis aimed to understand the functional impacts of altered splicing due to the toxic RNA.