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Mata, P.

Publications and source records attributed to Mata, P..

2 recordsLinked to original sources

Oligodendroglial dysfunction drives parvalbumin interneuron hypomyelination, circuit dysfunction and social deficits in an ASD model

Altered social behaviour in autism spectrum disorders (ASDs) has been linked to disruptions in neural circuit development, with emerging evidence implicating abnormal myelination. Yet how myelin development is affected and how this impacts circuit function remain unclear. Here, using the cntnap2-/- mouse model of ASD, we show that oligodendrocyte precursor cell (OPC) differentiation is transiently impaired during early postnatal development, resulting in delayed myelination in the somatosensory cortex and corpus callosum, altered myelin lipid composition, and impaired sheath wrapping, accompanied by transient microgliosis and increased oligodendrocyte apoptosis. Transcriptomic analysis reveals upregulation of interferon and immune-related pathways alongside downregulation of GABAergic synaptic genes in oligodendrocytes. These alterations converge on disrupted GABAergic interneuron to OPC communication, leading to hypomyelination and impaired maturation of parvalbumin-positive (PV) interneurons through a non-cell-autonomous mechanism, with selective deficits in PV function, excitation/inhibition balance and gamma oscillations. Early treatment with the promyelinating agents clemastine or pranlukast restores OPC differentiation, myelination, and PV maturation, rescues circuit dysfunction, and ameliorates social deficits and behavioural inflexibility. Our findings establish that transient developmental failure in OPC differentiation drives PV interneuron hypomyelination, linking oligodendroglial dysfunction to circuit-level and behavioural alterations in ASD, and highlight early myelin-targeted interventions as a potential therapeutic strategy.

neuroscience↗

IRF5 regulates microglial myelin clearance and cholesterol metabolism after demyelination

Interferon regulatory factor 5 (IRF5), a transcription factor highly involved in innate immunity that drives microglia/macrophage towards a pro-inflammatory state, has been associated to multiple sclerosis susceptibility but its role in MS pathogenesis is unknown. Here we analysed the role of IRF5 in multiple sclerosis animal models. Irf5-/- mice showed exacerbated damage in the chronic phase of experimental autoimmune encephalomyelitis (EAE) mice, despite an initial delay in its onset, as well as after lysolecithin injection into the spinal cord. Transcriptomic and lipidomic analysis evidence a role of this transcription factor in myelin metabolism and cholesterol homeostasis. Indeed, Irf5-/- mice showed an aberrant accumulation of myelin debris and lipidic structures, such as CE-containing lipid droplets and cholesterol crystals, suggesting that myelin-derived lipids are not properly processed. Cholesterol crystal accumulation leads to an aberrant inflammatory response, which block oligodendrocyte migration into the core of demyelinated lesion and remyelination. Pharmacologically facilitating cholesterol transport reduces lipid droplet accumulation and ameliorates EAE exacerbated damage in Irf5-/- mice. These results reveal for the first time the role of Irf5, a transcription factor necessary to orchestrate the immune responses, in phagocytes lipid metabolism which could be pivotal in regenerative responses such as remyelination.

neuroscience↗