It is also interesting to note that in a recently generated ADTKD-MUC1 mouse model that recapitulates a frame shift mutation in Muc1 (Muc1-fs mice), ATF6 is the prominent UPR branch that regulates ER stress response in response to the MUC-1 protein aggregates while with minimal transcriptional changes in the other two branches36. This evidence concerns the gene ATF6 and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.