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EGF responses in the CNS range from proliferation and survival to maturation of neurons and neuronal precursors 6, 7.
Depolarizing GABA is required for normal brain development, as it contributes to the morphological maturation of neurons [ 11], and neuronal circuits [ 12,13].
The expression of HuD coincides with the earliest stages of neuronal differentiation and is maintained through the maturation of neurons (Okano and Darnell, 1997; Wakamatsu and Weston, 1997; Clayton et al., 1998; Bolognani et al., 2007a; Hambardzumyan et al., 2009).
We show that iPSC-derived neurons and cardiomyocytes from FRDA patients maintain GAA repeat expansions of pathological size and express low levels of frataxin compared with control cells, resulting in neuronal and cardiac mitochondrial abnormalities and delayed functional maturation of neurons.
We do not see defects in neurogenesis and commitment to neuronal lineages, but find a dramatic defect in the later maturation of neurons after their birth.
Because of the observed delay in the maturation of neurons overexpressing miR-34a, we next analysed the expression of Doublecortin (DCX), a neuronal cytoskeleton protein that is critical for maturation and establishment of neuronal morphology.
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During the process of brain maturation, millions of neurons undergo apoptosis, yet leave barely a trace thanks to vigilant corpse removal attributable largely to microglia [ 126].
Cells exhibited typical electrophysiological features of immature neurons, consistent with the slow maturation of human neurons.
We discuss how mechanisms of chromatin regulation help to orchestrate the transcriptional programs that underlie the maturation of developing neurons and the plasticity of adult neurons.
Neurogenesis includes self-renewal and fate specification of neural stem cells, migration and maturation of young neurons, and functional integration of new neurons into the neural circuitry [ 1].
This pattern of expression suggests that MeCP2 is involved in the maturation of existing neurons rather than the development of new neurons from precursor cells.
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Justyna Jupowicz-Kozak
CEO of Professional Science Editing for Scientists @ prosciediting.com