Figure 5.

Traffic-deficient PMP22_L16P mutant generates Gd³⁺-sensitive, DVF-induced whole-cell currents in tsA201 cells. Representative whole-cell currents recorded from tsA201 cells transiently expressing PMP22_WT (A) or PMP22_L16P (B) exposed to +divalents (left, +Ca²⁺/Mg²⁺), divalent-free (center, −Ca²⁺/Mg²⁺), or divalent-free plus 10 μm Gd³⁺ (right) solutions. C, average IVs for Gd³⁺-sensitive DVF-induced currents measured from cells expressing PMP22_WT (♢, n = 11) or PMP22_L16P (♦, n = 14). D, concentration-response curves for Gd³⁺ block of DVF-induced currents recorded from tsA201 cells transiently expressing PMP22_WT (♢) or PMP22_L16P (♦). Solid lines represent data fit with Hill equation. n ≤5 for all concentrations.