Unlike GRP78, the upregulation of CHOP could be found only in ADTKD-UMOD cases, not in other CIN patients, and there were also definite colocalization of CHOP with uromodulin, suggesting that the activation of CHOP is specific in the pathogenesis of ADTKD-UMOD. The gene discussed is HSPA5; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.