Table 2.
ERAD components involved in targeting to the UPS
| Protein (human) | Other names | Protein (yeast) | Function | Ub-related domains | Other domains | L | M | C | TM |
|---|---|---|---|---|---|---|---|---|---|
| Substrate recognition and targeting | |||||||||
| SEL1 | Hrd3p | Substrate binding and recruitment | Sel1-like (12) | X | X | X | T1 | ||
| INSIG1, INSIG2 | Substrate binding and recruitment | X | X | X | 2 | ||||
| BAP31 | Substrate binding and recruitment | X | X | X | 3 | ||||
| ERLIN1, ERLIN2 | Substrate binding and recruitment | X | X | X | 4 | ||||
| OS-9 | Yos9p | Glycan binding | MRH | X | – | ||||
| XTP3-B | Glycan binding | MRH(2) | X | – | |||||
| ERMan1 | Mns1p | Glycan trimming | X | – | |||||
| EDEM1, EDEM2, EDEM3 | Htm1p | Glycan trimming | X | – | |||||
| PDI | P4HB | Disulfide-bond rearrangement | X | – | |||||
| BiP | GRP78 | Kar2p | Chaperone Hsp70 | X | – | ||||
| Hsp70 | Ssa1p | Chaperone Hsp70 | X | – | |||||
| GRP94 | gp96 | Chaperone Hsp90 | X | – | |||||
| ERdj4 | Chaperone Hsp40 | J | X | – | |||||
| ERdj5 | DNAJC10 | Scj1p | Chaperone Hsp40 | J, Trx | X | – | |||
| DNAJB12 | Chaperone Hsp40 | J | X | X | T2 | ||||
| Retrotranslocation and dislocation | |||||||||
| Derlin1, Derlin2, Derlin3 | Der1p, Dfm1p | Rhomboid pseudoprotease | SHPa | X | X | X | 6 | ||
| UBAC2 | Dsc2p | Rhomboid pseudoprotease | UBAb | X | X | X | 6 | ||
| RHBDL4 | Rhomboid protease | UIMb | VBMa | X | X | X | 6 | ||
| p97/VCP | Cdc48p | AAA+ ATPase | AAA | X | – | ||||
| TorsinA | DYT1 | AAA+ ATPase | AAA | X | – | ||||
| UbxD2 | Erasin | p97/VCP adaptor | UBX | X | X | MA | |||
| UbxD8 | ETEA | Ubx2p | p97/VCP adaptor | UBAb, UBX | X | X | MA | ||
| VIMP | SelS | p97/VCP adaptor | VIMa | X | X | T2 | |||
| Ufd1 | Ufd1p | p97/VCP adaptor | UT3b | BS1a | X | – | |||
| Npl4 | Npl4p | p97/VCP adaptor | NZFb, UBX | X | – | ||||
| NGly1 | PNGase1 | Png1p | Deglycosylation | PUBa, PAW | X | – | |||
| Substrate delivery to the proteasome | |||||||||
| Ubiquilin-1 | Dsk2p | Shuttling factor | UBL, UBAb | X | – | ||||
| hHR23A, hHR23B | Rad23p | Shuttling factor | UBL, UBAb | X | – | ||||
| DNAJB2 | HSJ1 | Shuttling factor | UIMb (2) | J | X | X | GG | ||
| Ubl4A | GET5 | Shuttling factor | UBL | X | – | ||||
| Trc35 | GET4 | Shuttling factor | X | – | |||||
| Bag6 | BAT3, Scythe | Shuttling factor, chaperone | UBL | X | – | ||||
| SGTA | Shuttling factor, chaperone | UBL binding domain | X | – | |||||
Common and alternate mammalian ERAD-component names are given, along with yeast orthologs. Putative function within ERAD is shown, in addition to ubiquitin-related and other domains (numbers of domains are shown in parentheses). Cellular exposure to ER lumen (L), ER membrane (M) or cytoplasm (C) is indicated by ‘X’. Links with the ER membranes are listed under TM: numbers, polytopic TM domains; T1, type I; T2, type II; MA, membrane associated; GG, geranylgeranylated.
a
p97/VCP-binding domain.
b
Ubiquitin-binding domain.