FH and hereditary leiomyomatosis and renal cell cancer: To determine whether the increase in urea cycle metabolites observed in FH1KO kidneys was recapitulated in FH mutant tumors, we used CE-TOFMS and IPA to analyze the metabolome of normal kidney (n = 2) and type 2 papillary tumors (n = 3) from two HLRCC patients (Table S1).