Imprecise excision of the progenitor insertion, deleting all transmembrane domains including the potassium selective pore region of the KCNQ channel.
KCNQ370 mutant larvae take 1-2 days longer to develop, a reduced number of animals eclose, and the mean lifespan of females is reduced by 20-30%. Mutant adults show a drastically increased incidence of pacing-induced cardiac dysfunction compared to age-matched controls, and the elevated failure rates observed in young mutant flies does not increase further with age.
Semi-intact KCNQ370 fly heart preparations from young (1-3 week old) flies exhibit severely nonrhythmic beating patterns, and the incidence of arrhythmia increases more rapidly with age compared to control preparations. A dramatic age-dependent increase of the heart period (defined as the length of time between the ends of two consecutive diastolic intervals) compared with age-matched controls is also observed, and both diastolic intervals and systolic intervals show age-dependent increases in length.
KCNQ370 is rescued by KCNQUAS.cOa/Scer\GAL4how-24B
Expression of KCNQScer\UAS.cOa under the control of Scer\GAL4how-24B in a KCNQ370 mutant background rescues the increased pacer-induced cardiac dysfunction rates and alterations in rhythmicity to levels comparable to those observed in control animals.