In CRS-4, despite high levels of mitochondrial dysfunction in cardiomyocytes, dysregulation of the PINK1/Parkin system, for reasons yet to be fully elucidated, prevents mitophagy and so promotes activation of mitochondrial cGAS-STING, NF-κB signalling, and subsequent LVH. The gene discussed is STING1; the disease is craniosynostosis 4.