UQCRFS1 and GRACILE syndrome: All other analyzed mitochondrial proteins (NDUFA9 for complex I, SDHA for complex II, Core1 for complex III, and COX1 for complex IV) were unaffected (Supplementary Figure S5A–D), indicating that the loss of RISP is replicated in the iPSC-derived hepatocyte and kidney tissue models of the GRACILE syndrome.