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Tuberous Sclerosis Complex as Disease Model for Investigating mTOR-Related Gliopathy During Epileptogenesis

Till S Zimmer1, Diede W M Broekaart1, Victoria-Elisabeth Gruber2

  • 1Department of (Neuro)Pathology, Amsterdam Neuroscience, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands.

Frontiers in Neurology
|October 12, 2020
PubMed

Insights

Tuberous sclerosis complex (TSC) involves brain abnormalities due to mTOR pathway issues. This review highlights how glial cell dysfunction contributes to epilepsy and neurodevelopmental disorders in TSC.

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Tuberous sclerosis complex (TSC) is a genetic disorder characterized by mammalian target of rapamycin (mTOR) pathway dysregulation.
  • TSC causes brain pathology, leading to epilepsy, autism, and intellectual disability.
  • Previous research has primarily focused on neuronal aspects of TSC.

Purpose of the Study:

  • To review the role of glial cell dysfunction in Tuberous Sclerosis Complex (TSC).
  • To explore the contribution of glial cells to epileptogenesis and neurodevelopmental comorbidities in TSC.
  • To discuss potential therapeutic strategies targeting glial abnormalities in TSC and related disorders.

Main Methods:

  • Literature review focusing on glial cell involvement in TSC.
  • Analysis of neuropathological hallmarks and functional changes in glial cells (astrocytes, oligodendrocytes, microglia).
  • Examination of interglial crosstalk and mTOR hyperactivity in the context of TSC.

Main Results:

  • Neuropathology in TSC involves significant morphological and functional changes in various glial cell types.
  • Glial cell dysfunction and their crosstalk are implicated in the mechanisms of epilepsy and cognitive/behavioral comorbidities in TSC.
  • mTOR hyperactivity in glial cells contributes to epileptogenesis.

Conclusions:

  • Glial cell dysfunction is a critical, yet understudied, component of Tuberous Sclerosis Complex (TSC) pathophysiology.
  • Understanding glial roles in TSC offers insights into mTOR-associated neurodevelopmental disorders.
  • Targeting glial abnormalities presents a promising avenue for novel therapeutic approaches in TSC.
Keywords:
astrocyteepilepsyepileptogenesisgliamammalian target of rapamycin (mTOR)microgliaoligodendrocytetuberous sclerosis (TSC)

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