Figure 4: Regulation of TRPML1 mutant isoforms by pH and additive effect between endogenous and synthetic TRPML1 ligands.
From: A small molecule restores function to TRPML1 mutant isoforms responsible for mucolipidosis type IV
(a) Similar to the WT channel activity of TRPML1 mutant isoform F408Δ is enhanced by low pH. (b) Lack of effect of low pH on F465L. (c) Bar diagram summarizing data at −200 mV from experiments shown in a,b. (d,e) Additive effect of PI(3,5)P2 and SF-22 on WT TRPML1 currents in lysosomes (d) and respective time course of current shown in d (e). (f,g) Lack of additive effect after co-application of SF-22 and MK6-83 (f) and respective time course of current shown in f (g). (h) Bar diagram summarizing data from experiments shown in d,g. ***P<0.001, **P<0.01; n=number of experiments. Data are presented as mean±s.e.m.
