TMEM70 deficiency is a mitochondrial genetic condition that display a detrimental impact on cardiac function. It participates in the assembly of ATP synthase, which is responsible for most energy generation provided by mitochondria. Patients with TMEM70 mutations have a high incidence of cardiac disease and early-onset death. Unfortunately, therapeutic interventions remain elusive, and are limited to symptomatic and supportive care. The researchers suggest the chemical AMPK modulation as a potential therapeutic strategy. By using CRISPR-engineered TMEM70-deficient iPSC lines, it could be possible to mimic cardiac features seen in the clinic and restore aberrant mitochondrial features previously studied in the model. The project presents a valuable opportunity to study innovative therapies for this devastating condition in a preclinical setting.