Likewise, the overexpression of endogenous MISTRH correlating with loss of MISTR1 (NDUFA4) protein (Fig 6D) had been observed in clear cell Renal Cell Carcinoma (ccRCC) tumor samples and ccRCC cell lines [73], a disease characterized by hyperactive HIF signaling [47], but up-regulation of MISTRAV and the requirement of HIF in the regulation of this newly proposed circuit had not been tested (Fig 6D). Here, COXFA4L3 is linked to clear cell renal carcinoma.