It was further verified by coimmunostaining for LONP1 and CD31, which is a specific marker of vascular endothelial cell (Fig. 1D), and the results showed that LONP1 was downregulated by 35 % in pediatric CKD samples, with inter-patient variability (27 %–44 % range) likely reflecting disease heterogeneity (Supplementary Fig. 1A and 1B). Here, LONP1 is linked to chronic kidney disease.