Figure 8. C277Y-induced changes in absolute open probabilities and unitary current amplitudes predict dramatically reduced chloride conductances in myotonic muscle fibres.

A, voltage dependences of instantaneous current amplitudes (same data as in Fig. 2D and F), normalized to unitary protopore current amplitudes obtained from single channel recordings at −100 mV (Fig. 7D). B, voltage dependences of absolute open probabilities of WT and C277Y hClC-1 at physiological pH. C, comparison of WT and C277Y steady-state current amplitudes displayed as mean current amplitude per single protopore.