Endoplasmic reticulum and Golgi stress in microcephaly
Microcephaly is a neurodevelopmental condition characterized by a small brain size associated with intellectual deficiency in most cases and is one of the most frequent clinical sign encountered in neurodevelopmental disorders. It can result from a wide range of environmental insults occurring durin...
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doaj-f2106d1978ef4a8da33b360054b255bd2020-11-25T01:22:05ZengShared Science Publishers OGCell Stress2523-02042019-10-0131236938410.15698/cst2019.12.206Endoplasmic reticulum and Golgi stress in microcephalySandrine Passemard0Franck Perez1Pierre Gressens2Vincent El Ghouzzi3Université de Paris, NeuroDiderot, Inserm, F-75019 Paris, France.Institut Curie, PSL Research University, CNRS, UMR144, Paris, France.Université de Paris, NeuroDiderot, Inserm, F-75019 Paris, France.Université de Paris, NeuroDiderot, Inserm, F-75019 Paris, France.Microcephaly is a neurodevelopmental condition characterized by a small brain size associated with intellectual deficiency in most cases and is one of the most frequent clinical sign encountered in neurodevelopmental disorders. It can result from a wide range of environmental insults occurring during pregnancy or postnatally, as well as from various genetic causes and represents a highly heterogeneous condition. However, several lines of evidence highlight a compromised mode of division of the cortical precursor cells during neurogenesis, affecting neural commitment or survival as one of the common mechanisms leading to a limited production of neurons and associated with the most severe forms of congenital microcephaly. In this context, the emergence of the endoplasmic reticulum (ER) and the Golgi apparatus as key guardians of cellular homeostasis, especially through the regulation of proteostasis, has raised the hypothesis that pathological ER and/or Golgi stress could contribute significantly to cortical impairments eliciting microcephaly. In this review, we discuss recent findings implicating ER and Golgi stress responses in early brain development and provide an overview of microcephaly-associated genes involved in these pathways.http://www.cell-stress.com/researcharticles/2019a-passemard-cell-stress/golgi apparatusendoplasmic reticulumstressuprcorticogenesisprimary microcephalygolgipathies |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sandrine Passemard Franck Perez Pierre Gressens Vincent El Ghouzzi |
spellingShingle |
Sandrine Passemard Franck Perez Pierre Gressens Vincent El Ghouzzi Endoplasmic reticulum and Golgi stress in microcephaly Cell Stress golgi apparatus endoplasmic reticulum stress upr corticogenesis primary microcephaly golgipathies |
author_facet |
Sandrine Passemard Franck Perez Pierre Gressens Vincent El Ghouzzi |
author_sort |
Sandrine Passemard |
title |
Endoplasmic reticulum and Golgi stress in microcephaly |
title_short |
Endoplasmic reticulum and Golgi stress in microcephaly |
title_full |
Endoplasmic reticulum and Golgi stress in microcephaly |
title_fullStr |
Endoplasmic reticulum and Golgi stress in microcephaly |
title_full_unstemmed |
Endoplasmic reticulum and Golgi stress in microcephaly |
title_sort |
endoplasmic reticulum and golgi stress in microcephaly |
publisher |
Shared Science Publishers OG |
series |
Cell Stress |
issn |
2523-0204 |
publishDate |
2019-10-01 |
description |
Microcephaly is a neurodevelopmental condition characterized by a small brain size associated with intellectual deficiency in most cases and is one of the most frequent clinical sign encountered in neurodevelopmental disorders. It can result from a wide range of environmental insults occurring during pregnancy or postnatally, as well as from various genetic causes and represents a highly heterogeneous condition. However, several lines of evidence highlight a compromised mode of division of the cortical precursor cells during neurogenesis, affecting neural commitment or survival as one of the common mechanisms leading to a limited production of neurons and associated with the most severe forms of congenital microcephaly. In this context, the emergence of the endoplasmic reticulum (ER) and the Golgi apparatus as key guardians of cellular homeostasis, especially through the regulation of proteostasis, has raised the hypothesis that pathological ER and/or Golgi stress could contribute significantly to cortical impairments eliciting microcephaly. In this review, we discuss recent findings implicating ER and Golgi stress responses in early brain development and provide an overview of microcephaly-associated genes involved in these pathways. |
topic |
golgi apparatus endoplasmic reticulum stress upr corticogenesis primary microcephaly golgipathies |
url |
http://www.cell-stress.com/researcharticles/2019a-passemard-cell-stress/ |
work_keys_str_mv |
AT sandrinepassemard endoplasmicreticulumandgolgistressinmicrocephaly AT franckperez endoplasmicreticulumandgolgistressinmicrocephaly AT pierregressens endoplasmicreticulumandgolgistressinmicrocephaly AT vincentelghouzzi endoplasmicreticulumandgolgistressinmicrocephaly |
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1725127855139979264 |