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- W2034348981 abstract "In previous studies we showed that transcomplementation of DF508 by truncated CFTR allows DF508 to progress to the cell surface. Here we study whether D27– 264 CFTR which is missing the first 4 transmembrane segments can also rescue DF508 chloride channel function. Whole cell currents were recorded from CHO cells expressing wt, DF508, D27–264, or both D27–264 and DF508 CFTR. Currents were activated by cpt-cAMP and Forskolin. Importantly no current was generated from unrescued DF508 alone, or from D27–264 alone. Only when both were added did we observe significant whole cell currents which reached to a level near to that of wtCFTR. The currents measured only when both are cotransfected could have originated from either from DF508 or D27–264 CFTR. To determine which one generated the currents, we utilized a CFTR conduction mutant, S341A, which alters the conductance of CFTR. S341A/ wtCFTR showed drastically reduced currents even though the protein was highly expressed. When we studied the double mutant, S341A/DF508 we noted expression of B band but no detectable chloride currents. When we cotransfected S341A /DF508 with D27–264 CFTR we did detect CFTR protein but the choride currents were dramatically reduced showing that the currents observed when DF508 and D27–264 CFTR are cotransfected were generated by rescued DF508. Immunoprecipitation studies showed that D27–264 can bind to DF508 CFTR. Our data show that transcomplementation with D27–264 can rescue both trafficking and chloride channel function of DF508 CFTR most likely via a bimolecular interaction. WS15.6 Upregulation of MicroRNA-148b in cystic fibrosis like lung disease of bENaC-overexpressing mice R. Agrawal1,2,3, M. Castoldi2,4, S. Altamura2,4, M.U. Muckenthaler2,4, M.A. Mall1,2,3. 1University of Heidelberg, Division of Paediatric Pulmonology and Cystic Fibrosis Center, Department of Paediatric III, Heidelberg, Germany; 2European Molecular Biology Laboratory and University of Heidelberg, Molecular Medicine Partnership Unit, Heidelberg, Germany; 3Translational Lung Research Center, Department of Translational Pulmonology, Heidelberg, Germany; 4University of Heidelberg, Department of Paediatric Oncology, Haematology and Immunology, Heidelberg, Germany" @default.
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- W2034348981 date "2012-06-01" @default.
- W2034348981 modified "2023-09-26" @default.
- W2034348981 title "WS15.6 Upregulation of MicroRNA-148b in cystic fibrosis like lung disease of ßENaC-overexpressing mice" @default.
- W2034348981 doi "https://doi.org/10.1016/s1569-1993(12)60108-8" @default.
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