Toward cell specificity in SCA1.

Transcriptional dysregulation appears as an emerging and unifying pathogenic mechanism in polyQ neurodegenerative disorders such as Spinocerebellar ataxias and Huntington's disease. It is unclear how cell death specificity occurs in these diseases. In this issue of Neuron, link polymerase II, a general component of the transcriptional machinery, to PQBP-1, a cerebellar enriched protein, thus providing insight into the selectivity of neuronal death in SCA1.