ALPK3 and Onset: In the homozygous setting, the mouse model recapitulates observations of clinical reports of biallelic carriers of ALPK3tv, leading to severe, often lethal, paediatric onset cardiomyopathy8,11,12: The homozygous Alpk3 K201X mice have impaired systolic function, dilatation and hypertrophy; we found their phenotype indistinguishable from Alpk3 knockout mice.