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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
Summary
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.
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