Our understanding of endoplasmic reticulum (ER) stress, the unfolded protein response (UPR) and ER stress-induced autophagy in the kidney has increased — using the presence of ER chaperones in the urine as a biomarker of renal ER stress, and using pharmacological agents that improve protein folding or induce the expression of chaperones, may aid in the diagnosis and treatment of kidney disease, respectively.