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.
Copyright © 2018. Published by Elsevier B.V.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Brain / embryology*
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Brain / metabolism
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Cell Differentiation / genetics
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Cell Movement / genetics
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Cerebral Cortex / metabolism
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Embryonic Development
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Facies
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Gene Expression Regulation, Developmental / genetics
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Hippocampus / metabolism
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Hirschsprung Disease
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Homeodomain Proteins / genetics
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Humans
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Intellectual Disability
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Microcephaly
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Neurogenesis / physiology
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Neuroglia / metabolism
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Neurons / metabolism
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Repressor Proteins / metabolism
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Stem Cells / physiology
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Transcription Factors / metabolism
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Zinc Finger E-box Binding Homeobox 2 / genetics*
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Zinc Finger E-box Binding Homeobox 2 / metabolism*
Substances
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Homeodomain Proteins
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Repressor Proteins
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Transcription Factors
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ZEB2 protein, human
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Zinc Finger E-box Binding Homeobox 2