DESIGNING TOOLS FOR EXACERBATING DYSTROPHIC PHENOTYPES IN ZEBRAFISH

Muscular dystrophy comprises a heteroge - neous group of genetic disorders that are characterized by progressive and irreversible degeneration of muscle mass. Each of the disrupted genes distinctively affects the gene expression of one component of the two pro - tein complexes in the muscle cell membrane: the Inte - grin-associated complex or Dystrophin-associated gly - coprotein complex. The latter links the Extracellular Matrix to the muscle internal actin cytoskeleton, pre - venting fiber detachment during muscle contraction and its malfunction leads to many different diseases. One of them results from a deficiency in the glycosylation of dystroglycan, leading laminin to fail to bind. As an ex - ample, Candyfloss (LAMA2) Zebrafish has shown that fibers detach naturally as the result of basement mem - brane failure, therefore the hypothesis is that a similar phenomenon would be observed when exacerbated fish using a small electroshock. Fish are conventionally elec - trocuted using a S88 Grass Stimulator, which is not effi - cient, since it electrocutes only one fish at a time. For this reason, we conducted studies to develop a new de - vice (Thomasi - Pires Lid, TPL) with a design inspired on a 48 well plate. Not only does the TPL enable the electrocution of 48 fish at a time, but it can also be used to image the fish and analyze the swimming assay on the ZebraBox, improving productivity.