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Role of Zeb2/Sip1 in neuronal development
Ekaterina Epifanova1, Alexey Babaev2, Andrew G Newman3
1Institute of Cell Biology and Neurobiology, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany; Lobachevsky State University of Nizhny Novgorod, Gagarina ave 23, 603950 Nizhny Novgorod, Russia.
Brain Research
|September 30, 2018
Summary
Zinc finger E-box binding homeobox 2 (Zeb2) is crucial for brain development. Mutations in Zeb2 cause Mowat-Wilson syndrome, leading to severe neurodevelopmental defects and Hirschsprung
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- The transcription factor Zeb2 (Sip1, Zfhx1b) is vital for neuronal development.
- Mutations in Zeb2 are linked to Mowat-Wilson syndrome, a condition characterized by Hirschsprung's disease and severe neurodevelopmental deficits.
Purpose of the Study:
- This review focuses on the molecular pathways regulated by Zeb2.
- It aims to elucidate how Zeb2 dysfunction leads to brain development disorders.
Main Methods:
- Literature review of studies on Zeb2 function in neuronal development.
- Analysis of molecular pathways downstream of Zeb2.
- Examination of genetic mutations and their phenotypic consequences.
Main Results:
- Zeb2 acts as a dual regulator, functioning as both a transcriptional repressor and activator.
- Zeb2 controls a broad spectrum of genes essential for various aspects of neuronal development.
- Dysregulation of Zeb2's downstream targets contributes to neurodevelopmental abnormalities.
Conclusions:
- Zeb2 plays a critical role in ensuring proper brain development.
- Understanding Zeb2's molecular pathways is key to addressing neurodevelopmental disorders like Mowat-Wilson syndrome.
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