In this study, we examined whether mucociliary clearance differed between cystic fibrosis (CF) knockout mice and wildtype controls. Additionally, we investigated whether infection with Pseudomonas aeruginosa, a common pathogen in the CF lung, affected this important host defence mechanism. Ciliary beat frequency (fcb) and particle transport (PT) were recorded using an in vitro lung explant prep...

Objective. To study the clinical manifestations of W1282R variant, functional activity cystic fibrosis transmembrane conductance regulator (CFTR) channel, and effectiveness CFTR modulator therapy in patients with variant. Materials methods included medical records, intestinal current measurement method (ICM) organoids (IO). Results. The c.3844T>C (p.Trp1282Arg, W1282R) variant refers to miss...

Cystic fibrosis (CF) is an autosomal recessive disorder that afflicts 72000 people world wide. It caused by over 2000 mutations to anion channel, the cystic transmembrane conductance regulator (CFtR) of which Over 300 present disease phenotype. We aim characterise all CF causing CFTR missense in Australian population using molecular dynamics modeling. Here we progress toward this goal presentin...

In protein redesign one of the main challenges is the ability to evaluate many different conformations of the redesigned protein in order to determine the best structure. One approach to this problem is to provably get rid of bad protein conformations using dead-end elimination (DEE). DEE was first developed for application to protein structure evaluation by Desmet et al. [1]. Since then there ...

We have used freeze-fracture electron microscopy to examine the oligomeric structure and molecular asymmetry of integral plasma membrane proteins. Recombinant plasma membrane proteins were functionally expressed in Xenopus laevis oocytes, and the dimensions of their freeze-fracture particles were analyzed. To characterize the freeze-fracture particles, we compared the particle cross-sectional a...