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What can Drosophila tell us about serpins, thrombosis and dementia?
Author(s) -
Carrell Robin,
Corral Javier
Publication year - 2004
Publication title -
bioessays
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.175
H-Index - 184
eISSN - 1521-1878
pISSN - 0265-9247
DOI - 10.1002/bies.10407
Subject(s) - drosophila (subgenus) , serpin , dementia , phenotype , drosophila melanogaster , mutation , biology , disease , genetics , protease , medicine , pathology , gene , biochemistry , enzyme
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. BioEssays 26:1–5, 2004. © 2003 Wiley Periodicals, Inc.