<p>Neurofibromatosis type 1 (NF1) is among the most common neurogenetic disorders and is associated with an increased risk of developing tumors in the nervous system. Additionally, up to 80% of patients with NF1 experience neurological complications, including deficits in attention, memory, and executive function. Significant effort has been dedicated to studying how <i>NF1</i> mutations autonomously dysregulate neuronal function. Increasing evidence indicates that <i>NF1</i> mutations also dysregulate the oligodendroglial lineage that contributes to neurological issues in NF1. Here, we summarize our current understanding of how <i>NF1</i> mutations impact the oligodendroglial lineage homeostasis and plasticity. We also discuss gaps in knowledge, potential therapeutic strategies, and future directions.</p>

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Current states in understanding oligodendroglia-mediated neurological issues in neurofibromatosis type 1 (NF1)

  • Benjamin E Aghoghovwia,
  • Cheng-En Shen,
  • Sabiha Bano,
  • Nandini Shyamala,
  • Alesandra Echeandia Marrero,
  • Khushboo Irshad,
  • Samer Sharafaldin,
  • Nicole M Brossier,
  • Yuan Pan

摘要

Neurofibromatosis type 1 (NF1) is among the most common neurogenetic disorders and is associated with an increased risk of developing tumors in the nervous system. Additionally, up to 80% of patients with NF1 experience neurological complications, including deficits in attention, memory, and executive function. Significant effort has been dedicated to studying how NF1 mutations autonomously dysregulate neuronal function. Increasing evidence indicates that NF1 mutations also dysregulate the oligodendroglial lineage that contributes to neurological issues in NF1. Here, we summarize our current understanding of how NF1 mutations impact the oligodendroglial lineage homeostasis and plasticity. We also discuss gaps in knowledge, potential therapeutic strategies, and future directions.