2 research outputs found
Fish models in prion biology: Underwater issues
13 páginas, 5 figuras, 1 tablaTransmissible spongiform encephalopathies (TSEs), otherwise known as prion disorders, are fatal diseases
causing neurodegeneration in a wide range of mammalian hosts, including humans. The causative agents –
prions – are thought to be composed of a rogue isoform of the endogenous prion protein (PrP). Beyond these
and other basic concepts, fundamental questions in prion biology remain unanswered, such as the
physiological function of PrP, the molecular mechanisms underlying prion pathogenesis, and the origin of
prions. To date, the occurrence of TSEs in lower vertebrates like fish and birds has received only limited
attention, despite the fact that these animals possess bona fide PrPs. Recent findings, however, have brought
fish before the footlights of prion research. Fish models are beginning to provide useful insights into the roles
of PrP in health and disease, as well as the potential risk of prion transmission between fish and mammals.
Although still in its infancy, the use of fish models in TSE research could significantly improve our basic
understanding of prion diseases, and also help anticipate risks to public health. This article is part of a Special
Issue entitled Zebrafish Models of Neurological Diseases.Thiswork is supported by a DFGgrant(MA 2525/2-1) andUniversity
of Konstanz funds awarded to EMT. ES, TS and AF are funded by EU
grants (QLK5-2002-00866 for ES, TS, AF and FOOD-CT-2004-506579 for
ES, TS)Peer reviewe