Function of PP2A-dependent signalling in early neurogenesis of vertebrates.

Spinocerebellar Ataxia 12 (SCA12) is a rare neurodegenerative disorder. Its origin is a mutation in PPP2R2B a subunit of the PP2A which leads to a CAG-repeat expansion and an increased gene expression (Holmes 2003). It has also been reported that along with SP1 and TFAp4, CREB1 can bind to the promotor region of the human PPP2R2B gene (Lin et al., 2010). Therefore indicating CREB1 could have an effect on the expression of PPP2R2B in SCA12. So far there are no in vivo studies showing PPP2A2B is involved in neuronal degeneration.

Here, we report the expression pattern of various neuronal markers in the neural tube on CREB1 and PP2A knock down embryos which show severe changes. In addition, stratification defects could also be observed in the retina. This implicates a connection with the published data.