Article
Positional dependence of non-native polar mutations on folding of CFTR helical hairpins.
Biochimica et biophysica acta - 1 Jan 2008
Wehbi Hania, Gasmi-Seabrook Geneviève, Choi Mei Y, Deber Charles M
Abstract excerpt
Mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) cause CF disease by altering the biosynthesis, maturation, folding and ion conductance of this protein. Our laboratory has focused on expression and structural analysis of the CFTR transmembrane (TM) domains using two-TM segments (i.e., helix-loop-helix constructs) which we term 'helical hairpins'; these represent the minimal model of...
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