Cholestasis, or impaired bile flow, is an important but poorly understood manifestation of liver disease. Two clinically distinct forms of inherited cholestasis, benign recurrent intrahepatic cholestasis (BRIC) and progressive familial intrahepatic cholestasis type 1 (PFIC1), were previously mapped
What can Drosophila tell us about serpins, thrombosis and dementia?
β Scribed by Robin Carrell; Javier Corral
- Publisher
- John Wiley and Sons
- Year
- 2003
- Tongue
- English
- Weight
- 228 KB
- Volume
- 26
- Category
- Article
- ISSN
- 0265-9247
No coin nor oath required. For personal study only.
β¦ Synopsis
Abstract
The validity of the fruitβfly 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 wellβstudied 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 26:1β5, 2004. Β© 2003 Wiley Periodicals, Inc.
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## Abstract ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 100 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a βFull Textβ option. The original article is trackable v