Abstract
The Olig genes encode members of the basic helix-loop-helix (bHLH) family of transcription factors. Olig1, Olig2, and Olig3 are expressed in both the developing and mature central nervous system (CNS) and strictly regulate cellular specification and differentiation. Extensive studies have established functional roles of Olig1 and Olig2 in directing neuronal and glial formation during different stages in development. Recently, Olig2 overexpression was implicated in neurodevelopmental disorders down syndrome (DS) and autism spectrum disorder (ASD) but its influence on cognitive and intellectual defects remains unknown. In this review, we summarize the biological functions of the Olig family and how it uniquely promotes cellular diversity in the CNS. This is followed up with a discussion on how abnormal Olig2 expression impacts brain development and function in DS and ASD. Collectively, the studies described here emphasize vital features of the Olig members and their distinctive potential roles in neurodevelopmental disease states.
Highlights
The central nervous system (CNS) is composed of various cell types that work synergistically for proper brain function
A significant reduction in myelin basic protein (MBP) and DM20/PLP was observed between E18.5 and P0 in heterozygotes compared to WT mice suggesting that a full dosage of Olig genes is required for the progression of oligodendrocyte differentiation (Zhou and Anderson, 2002)
It is well recognized that Olig1 and Olig2 have divergent, non-overlapping roles in cellular patterning and specification, mechanisms underlying dysfunctional Olig1 and Olig2 leading to aberrant cell identities and functions remain to be determined
Summary
Olig, and Olig are expressed in both the developing and mature central nervous system (CNS) and strictly regulate cellular specification and differentiation. Extensive studies have established functional roles of Olig and Olig in directing neuronal and glial formation during different stages in development. We summarize the biological functions of the Olig family and how it uniquely promotes cellular diversity in the CNS. This is followed up with a discussion on how abnormal Olig expression impacts brain development and function in DS and ASD. The studies described here emphasize vital features of the Olig members and their distinctive potential roles in neurodevelopmental disease states
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