Figure 1.

An updated model illustrating the relationship between an opening/closing cycle of the gate and ATP consumption in CFTR’ s NBDs. The trace on top represents an opening burst of WT-CFTR. The cartoon below shows transitions of gating states throughout the gating cycle. Notably, the C2 state is extremely stable and can last for ∼30 s before returning to the C1 state (Tsai et al., 2010b). C1, closed state with two separated NBDs; C2, partial NBD dimeric closed state; O1, prehydrolytic open state; O1′, post-hydrolytic open state with a full NBD dimer; X, the state with partial separated NBD dimer (Jih et al., 2012); X′, one ATP bound to the vacant ABP2 in state X. The red box encompasses the reentry pathway that may occur within each opening burst. This figure is adapted from Jih et al. (2012).

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