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Scheme of neuroinflammation and neurodegeneration in JNPL3 and TTBK1/JNPL3 mice. In JNPL3 mice, CD11c+ neuroprotective microglia accumulate in the gray matter of the ventral horn, thereby protecting from accelerated neurodegeneration. Conversely, in TTBK1/JNPL3 mice, the cell population is shifted from CD11c+ microglia to M1-skewed infiltrating NOS2+CD169+ monocytes, resulting in enhanced neuroinflammation and accelerated motor neuron loss.
