Obiettivo Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) affect the maturation and trafficking of the protein in the cell or cause dysfunction of this cell surface expressed chloride channel. To rescue the cells, compounds will be designed capable of correcting the processing and dysfunction of CFTR. In addition known interacting proteins and novel interacting proteins, identified during the proposed investigations will be characterized at the genetic, biochemical and electrophysiological level and their ability to compensate for or rescue the cells from dysfunction will be determined (in combination with selective compounds) . The thus identified protein network should contribute to the understanding of the clinical variation observed in affected sibs, the role of similar mutations in the frequent CF related diseases and could provide tools to diagnose, monitor and treat these diseases. Campo scientifico natural sciencesbiological sciencesbiochemistrybiomoleculesproteinsnatural sciencesbiological sciencesgeneticsmutation Programma(i) FP5-LIFE QUALITY - Specific Programme for research, technological development and demonstration on "Quality of life and management of living resources", 1998-2002 Argomento(i) 1.1.1.-7. - Chronic and degenerative diseases, cancer, diabetes, cardivascular diseases and rare diseases Invito a presentare proposte Data not available Meccanismo di finanziamento CSC - Cost-sharing contracts Coordinatore KATHOLIEKE UNIVERSITEIT LEUVEN Contributo UE Nessun dato Indirizzo 49,Herestraat 49 3000 LOUVAIN / LEUVEN Belgio Mostra sulla mappa Costo totale Nessun dato Partecipanti (7) Classifica in ordine alfabetico Classifica per Contributo UE Espandi tutto Riduci tutto Clondiag Chip Technologies GmbH Germania Contributo UE Nessun dato Indirizzo Loebstedter Strasse 103-105 07749 Jena Mostra sulla mappa Collegamenti Sito web Opens in new window Costo totale Nessun dato ERASMUS UNIVERSITEIT ROTTERDAM Paesi Bassi Contributo UE Nessun dato Indirizzo Dr Molewaterplein 50 3000 DR ROTTERDAM Mostra sulla mappa Costo totale Nessun dato EUROGENTEC SA Belgio Contributo UE Nessun dato Indirizzo Rue Bois Saint Jean 14 4102 OUGREE Mostra sulla mappa Costo totale Nessun dato HANNOVER MEDICAL SCHOOL Germania Contributo UE Nessun dato Indirizzo Carl-Neuberg-Strasse 1 30625 HANNOVER Mostra sulla mappa Costo totale Nessun dato KLINIKUM DER FRIEDRICH-SCHILLER-UNIVERSITAET JENA Germania Contributo UE Nessun dato Indirizzo Erlanger Allee 101 07747 JENA Mostra sulla mappa Costo totale Nessun dato UNIVERSITE DE POITIERS Francia Contributo UE Nessun dato Indirizzo Avenue du Recteur Pineau, 40 86022 POITIERS Mostra sulla mappa Costo totale Nessun dato UNIVERSITY OF BRISTOL Regno Unito Contributo UE Nessun dato Indirizzo University Walk BS8 1TD BRISTOL, CLIFTON Mostra sulla mappa Costo totale Nessun dato