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Cystic fibrosis: rescue of the function and of the processing of cftr mutants by pharmacological agents and by interacting proteins (CF-PRONET)

Objective

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.

Funding Scheme

CSC - Cost-sharing contracts

Coordinator

KATHOLIEKE UNIVERSITEIT LEUVEN
Address
49,Herestraat 49
3000 Louvain / Leuven
Belgium

Participants (7)

Clondiag Chip Technologies GmbH
Germany
Address
Loebstedter Strasse 103-105
07749 Jena
ERASMUS UNIVERSITEIT ROTTERDAM
Netherlands
Address
Dr Molewaterplein 50
3000 DR Rotterdam
EUROGENTEC SA
Belgium
Address
Rue Bois Saint Jean 14
4102 Ougree
HANNOVER MEDICAL SCHOOL
Germany
Address
Carl-neuberg-strasse 1
30625 Hannover
KLINIKUM DER FRIEDRICH-SCHILLER-UNIVERSITAET JENA
Germany
Address
Erlanger Allee 101
07747 Jena
UNIVERSITE DE POITIERS
France
Address
Avenue Du Recteur Pineau, 40
86022 Poitiers
UNIVERSITY OF BRISTOL
United Kingdom
Address
University Walk
BS8 1TD Bristol, Clifton