. Loss of poly(ADP-ribose) glycohydrolase causes progressive neurodegeneration in Drosophila melanogaster. Proc Natl Acad Sci U S A. 2004 Jan 6;101(1):82-6. PubMed.

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  1. Polyadenylation is key to a number of nuclear functions, notably the regulation of mRNA stability and DNA repair. The extensive accumulation of polyA in neurons of polyA glycohydrolase mutant flies suggests that there is a major effect on mRNA stability in the CNS. This paper raises the possibility that polyA metabolism may be disproportionately important for the regulation of gene expression in neurons, and that dysregulation of this process may contribute to neurodegenerative conditions. It would thus be of interest to examine the state of polyA metabolism in neurodegenerative diseases, particularly those with prominent nuclear lesions such as Huntington's disease.