Disruption of oxidative phosphorylation has previously been shown to lead to increases in ROS within cells, rodent models, and in patients with nephrolithiasis, and these increases in ROS have been hypothesized to contribute to renal cell damage, and consequently crystal retention and nephrocalcinosis.37, 41, 42, 43 To determine if the POLG2‐Tyr265Stop mutation increased ROS production, we assessed levels of ROS36 in plasma samples from Polg2+/+ and Polg2+/Y265X mice. This evidence concerns the gene POLG2 and nephrolithiasis.