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Endoplasmic reticulum and Golgi stress in microcephaly

Sandrine Passemard1,2, Franck Perez3, Pierre Gressens1,4

  • 1Université de Paris, NeuroDiderot, Inserm, F-75019 Paris, France.

Cell Stress
|December 14, 2019
PubMed

Insights

Microcephaly, a condition causing small brain size and intellectual deficiency, may stem from endoplasmic reticulum (ER) and Golgi stress impacting neuron production during early brain development.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Genetics

Background:

  • Microcephaly is a neurodevelopmental disorder linked to intellectual deficiency, often caused by environmental or genetic factors.
  • A common mechanism involves impaired cortical precursor cell division during neurogenesis, limiting neuron production.
  • Severe congenital microcephaly is associated with these neuronal deficits.

Purpose of the Study:

  • To review recent findings on endoplasmic reticulum (ER) and Golgi stress in early brain development.
  • To explore the hypothesis that ER/Golgi stress contributes to microcephaly.
  • To provide an overview of genes involved in these pathways and linked to microcephaly.

Main Methods:

  • Literature review of recent findings on ER and Golgi stress in neurodevelopment.
  • Analysis of studies linking cellular stress pathways to microcephaly.
  • Compilation of microcephaly-associated genes involved in ER/Golgi homeostasis.

Main Results:

  • Evidence suggests compromised cortical precursor cell division is a key mechanism in severe microcephaly.
  • Pathological ER and Golgi stress are hypothesized to significantly impair cortical development.
  • Several genes involved in ER/Golgi stress pathways are implicated in microcephaly.

Conclusions:

  • ER and Golgi stress responses play a crucial role in regulating cellular homeostasis and proteostasis during early brain development.
  • Dysregulation of these stress pathways may be a significant contributor to the pathogenesis of microcephaly.
  • Further research into ER/Golgi stress and associated genes is vital for understanding and potentially treating microcephaly.

Keywords:
Golgi apparatusUPRcorticogenesisendoplasmic reticulumgolgipathiesprimary microcephalystress

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