Neurogenetics Branch National Institute of Neurological Disorders and Stroke Bethesda, Maryland, USA kf@codon.nih.gov
The mutant proteins that cause polyglutamine disease bind CREB-binding protein (CBP), a key transcriptional co-activator for neuronal survival factors. This results in a loss of CBP-dependent transcription and may account for the neuronal degeneration associated with these diseases.