Both mutant and WT misfolded proAVP proteins undergo proteasomal degradation.[24, 31] The SEL1L‐HRD1 ERAD pathway degrades misfolded WT proAVP to prevent the formation of aberrant disulfide bonds between proAVP molecules.[24] Loss of Sel1L in AVP neurons causes ER retention and aggregation of proAVP and the development of a diabetes insipidus phenotype in mice. This evidence concerns the gene AVP and Central diabetes insipidus.