Cystic fibrosis (CF) is caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel. The most common CF mutation F508del produces a misfolded protein with three major defects: reduced protein processing; aberrant channel gating and thermo-instability. Low-temperature incubation partially rescues the plasma membrane expression of FS08del-CFTR. However, the channel is unstable and runs down within minutes when studied at 37°C in excised inside-out membrane patches. The aims of my research were to study wild-type and FS08del-CFTR channel behaviour at different temperatures and investigate thermoinstability of F508del-CFTR at the plasma membrane and effects of small-molecules.
| Identifer | oai:union.ndltd.org:bl.uk/oai:ethos.bl.uk:702448 |
| Date | January 2015 |
| Creators | Wang, Yiting |
| Publisher | University of Bristol |
| Source Sets | Ethos UK |
| Detected Language | English |
| Type | Electronic Thesis or Dissertation |
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