What can Drosophila tell us about serpins, thrombosis and dementia?

The validity of the fruit-fly as a model of human disease has been confirmed in a striking way by Green and colleagues.1 They show that the mutations causing a necrotic disease phenotype in Drosophila, precisely mirror those resulting in a group of well-studied but perplexing diseases in the human. These diseases, ranging from thrombosis to dementia, arise from mutations causing a conformational instability of serpin protease inhibitors. The findings provide clues as to the unusual severity and variable onset of such conformational diseases and demonstrate the potential of Drosophila as a model for their future study.