Role of Zeb2/Sip1 in neuronal development

Brain Res. 2019 Feb 15:1705:24-31. doi: 10.1016/j.brainres.2018.09.034. Epub 2018 Sep 25.

Abstract

Zeb2 (Sip1, Zfhx1b) is a transcription factor that plays essential role in neuronal development. Sip1 mutation in humans was shown to cause Mowat-Wilson syndrome, a syndromic form of Hirschprung's disease. Affected individuals exhibit multiple severe neurodevelopmental defects. Zeb2 can act as both transcriptional repressor and activator. It controls expression of a wide number of genes that regulate various aspects of neuronal development. This review addresses the molecular pathways acting downstream of Zeb2 that cause brain development disorders.

Keywords: Brain development; Cortex; Hippocampus; Mowat-Wilson syndrome.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Brain / embryology*
  • Brain / metabolism
  • Cell Differentiation / genetics
  • Cell Movement / genetics
  • Cerebral Cortex / metabolism
  • Embryonic Development
  • Facies
  • Gene Expression Regulation, Developmental / genetics
  • Hippocampus / metabolism
  • Hirschsprung Disease
  • Homeodomain Proteins / genetics
  • Humans
  • Intellectual Disability
  • Microcephaly
  • Neurogenesis / physiology
  • Neuroglia / metabolism
  • Neurons / metabolism
  • Repressor Proteins / metabolism
  • Stem Cells / physiology
  • Transcription Factors / metabolism
  • Zinc Finger E-box Binding Homeobox 2 / genetics*
  • Zinc Finger E-box Binding Homeobox 2 / metabolism*

Substances

  • Homeodomain Proteins
  • Repressor Proteins
  • Transcription Factors
  • ZEB2 protein, human
  • Zinc Finger E-box Binding Homeobox 2

Supplementary concepts

  • Mowat-Wilson syndrome