DES and Desminopathy: Indeed, the aggregate-prone CRYAB-R120G mutant protein is phosphorylated at serine 59 in the mouse myocardium in transgenic R120G mice (Supplemental Figure 3), confirming prior observations in cell culture (38), in a mouse model for proteotoxic cardiomyopathy that recapitulates human pathology with mislocalization of desmin to protein aggregates (18) to induce a desminopathy.