Since initially identified as a negative regulator of mitochondrial antiviral signaling (MAVS) protein, the functional significance of NLRX1 in biology and medicine has immensely expanded, revealing a crucial role of NLRX1 in a multitude of animal disease models in vivo, including cancer, virus infection, osteoarthritis, traumatic brain injury, inflammatory bowel disease, autoimmune encephalomyelitis, and COPD (reviewed in reference [Nagai‐Singer et al., 2019]). Here, NLRX1 is linked to glycogen storage disease VI.