VHL and Renal neoplasm: Interestingly, the phenomenon of CI loss has also been observed in several other kidney neoplasias, including FH-mutant type 2 papillary renal cell carcinomas (pRCC), renal oncocytomas driven by mitochondrial DNA mutations, and VHL-mutant clear cell renal cell carcinomas (ccRCC), which are characterized by suppression of oxidative mitochondrial metabolism (Courtney et al., 2018; Crooks et al., 2021; Mayr et al., 2008; Simonnet et al., 2003; Tomlinson et al., 2002).