The coding sequence of the endogenous sgll-RB isoform has been deleted, and cDNA sequence encoding the orthologous Hsap\PNPO gene has been inserted in its place. The Hsap\PNPO coding sequence contains a A98T nucleotide substitution, corresponding to the D33V amino acid replacement, a variant associated with epilepsy.
sgllKI.hPNPO.D33V/sgllKI.hPNPO.R95H adults show increased susceptibility (mortality) to sugar-only diet. These adults show hypoactivity (e.g. decreased walking distance and speed) under a FB or CYM conventional diet. These adults also show hypoactivity under a sugar-only diet if preceded by a FB, but not CYM, conventional diet. Under a sugar-only diet, dorsal longitudinal muscles show spontaneous seizure activity, manifested as high-frequency spike burst discharges.
sgllKI.hPNPO.D33V homozygous adults show hyperactivity (e.g. increased walking distance and speed) under a FB, but not CYM, conventional diet. These adults show hyperactivity (e.g. increased walking distance and speed) under a sugar-only diet if preceded by a FB, but not CYM, conventional diet. Under a sugar-only diet, dorsal longitudinal muscles show spontaneous grooming-associated activity patterns, with higher firing frequently than controls.
