KATP Channels in Zebrafish Cardiovascular System: A Model to Study Cantú Syndrome

February 2020 in “ Biophysical journal ”

Soma S. Singareddy, Helen I. Roessler, Conor McClenaghan, Rob C. Tryon, Gijs van Haaften, Colin G. Nichols

KATP channels Cantú Syndrome gain-of-function mutations cardiovascular complications excessive hair growth ventricular cardiomyocytes ATP sensitivity sulfonylurea inhibition

TLDR Zebrafish with mutations similar to human Cantú Syndrome have heart cells with altered channel properties, making them a good model to study the condition.

The document discusses a study on zebrafish (ZF) as a model to understand Cantú Syndrome (CS), a condition caused by gain-of-function mutations in genes encoding KATP channel subunits, leading to cardiovascular complications and excessive hair growth. The study generated ZF lines with mutations equivalent to those causing CS in humans and characterized KATP channel expression and function in ZF cardiovascular cells. Results showed that ZF ventricular cardiomyocytes (VCMs) express KATP channels with properties similar to mammalian ventricles, and the mutant channels exhibited altered ATP sensitivity and decreased sensitivity to sulfonylurea inhibition, confirming the presence of KATP channels similar to those in the mammalian cardiovascular system and validating the use of ZF as a model for studying CS.

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