Enhanced oligodendrocyte maturation and myelination in a mouse model of Timothy syndrome

Glia
Veronica T CheliPablo M Paez

Abstract

To study the role of L-type voltage-gated Ca++ channels in oligodendrocyte development, we used a mouse model of Timothy syndrome (TS) in which a gain-of-function mutation in the α1 subunit of the L-type Ca++ channel Cav1.2 gives rise to an autism spectrum disorder (ASD). Oligodendrocyte progenitor cells (OPCs) isolated from the cortex of TS mice showed greater L-type Ca++ influx and displayed characteristics suggestive of advanced maturation compared to control OPCs, including a more complex morphology and higher levels of myelin protein expression. Consistent with this, expression of Cav1.2 channels bearing the TS mutation in wild-type OPCs triggered process formation and promoted oligodendrocyte-neuron interaction via the activation of Ca++ /calmodulin-dependent protein kinase II. To ascertain whether accelerated OPC maturation correlated with functional enhancements, we examined myelination in the TS brain at different postnatal time points. The expression of myelin proteins was significantly higher in the corpus callosum, cortex and striatum of TS animals, and immunohistochemical analysis for oligodendrocyte stage-specific markers revealed an increase in the density of myelinating oligodendrocytes in several areas of the T...Continue Reading

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Citations

Apr 9, 2019·Glia·Chloé HabermacherNajate Benamer
Jun 22, 2019·Cellular and Molecular Neurobiology·Ming ZhangXianghui Zhao
Feb 23, 2020·Annual Review of Neuroscience·Pablo M Paez, David A Lyons
Jul 22, 2019·Experimental Biology and Medicine·Dan HanGuoliang Li
Jul 6, 2020·Pflügers Archiv : European journal of physiology·Andrea MarcantoniEmilio Carbone
Nov 5, 2019·Neuroscience·Vitor S AlvesFernando A Oliveira
Mar 13, 2021·Frontiers in Cellular Neuroscience·Dorien A Maas, María Cecilia Angulo
May 15, 2021·Orphanet Journal of Rare Diseases·Miriam KessiFei Yin
Jul 25, 2021·International Journal of Molecular Sciences·Federica CherchiElisabetta Coppi
Jan 5, 2022·Cell Regeneration·Xiaoxiang LuYangfei Xiang

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