Myotonia congenita-associated mutations in chloride channel-1 affect zebrafish body wave swimming kinematics.

ABSTRACT: Myotonia congenita is a human muscle disorder caused by mutations in CLCN1, which encodes human chloride channel 1 (CLCN1). Zebrafish is becoming an increasingly useful model for human diseases, including muscle disorders. In this study, we generated transgenic zebrafish expressing, under the control of a muscle specific promoter, human CLCN1 carrying mutations that have been identified in human patients suffering from myotonia congenita. We developed video analytic tools that are able to provide precise quantitative measurements of movement abnormalities in order to analyse the effect of these CLCN1 mutations on adult transgenic zebrafish swimming. Two new parameters for body-wave kinematics of swimming reveal changes in body curvature and tail offset in transgenic zebrafish expressing th

SUBMITTER: Cheng W 

PROVIDER: S-EPMC4118878 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature