Small-molecule VHL binderscan act as competitors to the native substrate HIF-1α and stabilizeHIF-1α levels, upregulating genes involved in the hypoxic response,consequently affecting hypoxia signaling.3,13 Thesuccessful development of VHL inhibitors has emphasized the importanceof VHL as a therapeutic target for the treatment of conditions thatoccur in anemia, ischemic, inflammatory, or mitochondrial diseases.3,10. Here, VHL is linked to inborn mitochondrial metabolism disorder.