The P101L mutation in Mmus\PrnpP101L.Scer\UAS.cMa corresponds to the P102L mutation in humans, which is associated with Gerstmann-Straussler-Scheinker syndrome patients.
Mouse gene.
Flies expressing of Mmus\PrnpP101L.Scer\UAS.cMa under the control of Scer\GAL4ChAT.7.4 (limited to the adult stages using Scer\GAL80ts.αTub84B and shifting the flies to the restrictive temperature at eclosion) display climbing defects. These phenotypes are more severe than when the wild type protein is expressed (Mmus\PrnpScer\UAS.cMa). Climbing behavior is restored when the flies are returned to the permissive temperature following a two-, three- or four-day pulse of expression. An increased proportion of flies show climbing ability following 10 days of recovery compared with those that only received the pulse.
Cholinergic neurons from adult brains expressing Mmus\PrnpP101L.Scer\UAS.cMa under the control of Scer\GAL4ChAT.7.4 exhibit reduced spontaneous activity compared with controls. The phenotype is more severe than when the wild type protein is expressed (Mmus\PrnpScer\UAS.cMa). Activity is restored when the flies are returned to the permissive temperature for 10 days following a two-, three- or four-day pulse of expression.