Fig. 1

C. elegans models of channelopathies show behavioral defects. a Schematic illumination of chimeric ion channels. The chimeric ion channels consist of transmembrane domains and their connecting loops of a human ion channel, and N-/C-terminus of its C. elegans homolog. The gain-of-function mutation was expected to alter cell excitability and consequently induce behavioral defects in transgenic worms. b Schematic illumination of generating animal models of channelopathies and strategy for drug screenings. Transgenic worms expressing chimeric ion channel 1 proteins will exhibit clear behavior defects. Drugs that alleviate the phenotypic defects could be inhibitors of the channel. Transgenic worms expressing chimeric ion channel 2 proteins will show no or weak behavior defects due to the reduced channel function, and drugs aggravate the worms’ behavioral defects are potential activators or functional correctors of the channel. c Microscopy images of wild-type N2 and hERG^{Chimera/A536W} transgenic worms. Scale bar, 200 µM. White arrows indicate C. elegans eggs. d, e Locomotive (d) and egg-laying (e) behaviors of of young adult wild-type N2 and hERG^{Chimera/A536W} transgenic worms. f Fluorescent images of worms expressing both mCherry and hERG^{chimera/A536W}::GFP proteins that were driven by the unc-103 promoter. Blue arrows indicate head neurons. Scale bar, 10 μm. All data shown are mean ± s.e.m. ***P < 0.001 (Student’s t-tests for d, e)