Finally, by using an arginine-depleting enzyme with US Food and Drug Administration (FDA) orphan drug status, pegylated arginine deiminase (ADI-PEG 20) [16], we demonstrated that blocking the conversion of arginine to argininosuccinate specifically reduces cell proliferation of FH-deficient cells, opening new opportunities for the treatment of FH-deficient renal cell cancer. Here, FH is linked to renal cell adenocarcinoma.