We display here that fumarate-mediated succination of ACO2 impairs its enzymatic activity inside a dose-dependent manner and that Fh1KO cells exhibit reduced aconitase activity
We display here that fumarate-mediated succination of ACO2 impairs its enzymatic activity inside a dose-dependent manner and that Fh1KO cells exhibit reduced aconitase activity. Our data provide evidence that succination, resulting from FH deficiency, focuses on and potentially alters the function of multiple proteins and may contribute?to the dysregulated metabolism observed in HLRCC. Abstract Graphical…