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. 2012 Dec 13;3:201. doi: 10.3389/fphar.2012.00201

Figure 4.

Figure 4

Step-wise CFTR folding pathway. (A) CFTR folding begins cotranslationally as individual domains are synthesized, and proceeds as domains assemble into a mature tertiary structure. (B) The ΔF508 mutation destabilizes NBD1 structure, interferes with the TMD1, TMD2, and NBD2 folding, and disturbs interactions between NBD1 and ICL4, compromising domain–domain assembly.