Three types of misfolded proteins, ER luminal (OS9), transmembrane (EDEM1, IRE1α and pre-BCR), and nuclear proteins (NRF2, PGC1β and BLIMP1), have been identified as Sel1L-Hrd1 ERAD substrates using ERAD-deficient mouse models. Substrates in bold (OS9 and IRE1α) have been tested in several different cell types while those in plain fonts have been tested in only one cell type. Substrate-related function is indicated in parentheses. Among all the substrates, the pre-BCR protein complex is unique in that it is consisted of heavy chain Igµ and light chains λ5 and VpreB.