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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.

Call for proposal

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Coordinator

KATHOLIEKE UNIVERSITEIT LEUVEN
EU contribution
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Address
49,Herestraat 49
3000 LOUVAIN / LEUVEN
Belgium

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Total cost
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Participants (7)