Schematic drawing the mechanisms underlying cell death of neural and oligodendrocyte progenitor cells in Ddx20-deficient central nervous system (IMAGE)

Niigata University

Schematic drawing the mechanisms underlying cell death of neural and oligodendrocyte progenitor cells in Ddx20-deficient central nervous system

Caption

In Ddx20-deficient cells, the SMN complex collapses due to destabilization of SMN protein, resulting in abnormal RNA splicing process (pink background). This leads to DNA damage and abnormal splicing of Mdm2 mRNA, and activation of the p53 pathway due to reduced p53 proteolysis, resulting in apoptosis (cell death) of neural progenitor cells and oligodendrocyte progenitor cells.

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Niigata University

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