Reflecting the activity of the ATF6 arm of the UPR, cysteine-rich with EGF-like domains 2 (CRELD2) is upregulated and secreted when renal cells are exposed to ER stressors and appears to be a potential urine ER stress biomarker in patients with kidney diseases associated with ER stress including ADTKD-UMOD and ischemic AKI38. Here, CRELD2 is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.