Doppel expression is regulated by the Brn-3a and Brn-3b transcription factors.

Doppel and the prion protein (PrP) are two related proteins involved in different aspects of neuronal degeneration. While a structural modification of PrP is necessary and sufficient for its toxic effect, the neurotoxicity of Doppel in the Purkinje cells of the cerebellum relies solely on its overexpression. Understanding the Doppel-related neurotoxicity thus involves the analysis of its developmental and transcriptional regulation. Here we report for the first time that Doppel is expressed in the embryonic neurons of mice dorsal root ganglia and spinal cord and that the closely related Brn-3a and Brn-3b transcription factors are involved in its transcriptional regulation.