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Review
. 2014 Jul 16;4(4):312-24.
eCollection 2014.

Role of SOX family of transcription factors in central nervous system tumors

Arlet M Acanda de la Rocha  1 , Nicolas Sampron  2 , Marta M Alonso  1 , Ander Matheu  3
Affiliations
Review

Role of SOX family of transcription factors in central nervous system tumors

Arlet M Acanda de la Rocha et al. Am J Cancer Res. .

Abstract

SOX genes are developmental regulators with functions in the instruction of cell fate and maintenance of progenitor's identity during embryogenesis. They play additional roles during tissue homeostasis and regeneration in adults particularly in the Central Nervous System (CNS). In the last years a growing number of evidences has shown that mutations and dysfunction of SOX factors are implicated in several human diseases, including a variety of cancers. In this review, we will summarize the current knowledge about SOX family in CNS tumors and their role in the origin and maintenance of the subpopulation of cancer stem cells in these tumors.

Keywords: CNS tumors; SOX; cell of origin; glioblastoma; glioma stem cell; oncogenic SOX2; therapy target.

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Figures

Figure 1
Figure 1
Glioma cell of origin and glioma stem cell (GSC) evolution. Normal cellular hierarchy comprises neural stem cells that progressively produce new stem cells and more restricted progenitor cells, finally yielding oligodendrocytes, astrocytes and neurons. Accumulation of genetic mutations in different cell types is sufficient to induce gliomas. These gliomas contain a population of GSCs with self-renewal capacity and ability to differentiate to all the lineages.
Figure 2
Figure 2
Balance between SOX members in the regulation of GSCs. Different SOX members display opposite functions in the regulation of GSCs. However, it remains elusive whether they are involved in the initiation of the glioma.
Figure 3
Figure 3
SOX2 functions in tissue homeostasis and cancer. SOX2 is emerging as a very complex factor with multiple functions. Here we include the most relevant for glioma.
See this image and copyright information in PMC

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