Table 1.

Mammalian ERAD genes and their predicted orthologs in C. elegansa

Mammalian geneWorm genePutative functionMutant availableMutation descriptionComment
EDEM1C47E12.3Glycosylhydrolase-47 familyok2898378 bp deletion
EDEM2F10C2.5Glycosylhydrolase-47 familytm6057563 bp deletionAffects >1 gene
OS9Y105E8A0.2Inspects client proteins for terminal α1,6 linked mannosetm5511517 bp deletion
TEB4F55A3.1DOA10 ligase componentgk218188Introduces stop codon mid-gene
ErasinZK353.8DOA10 ligase componenttm3247323 bp deletionTested
HRD1F55A11.3E3 ubiquitin ligasetm1743473 bp deletion + 38 bp insertion
HRDL-1F26E4.11E3 ubiquitin ligasegk281981 bp deletion
Derlin-1F25D7.1HRD ligase component; retrotranslocontm2839224 bp deletionTested
Derlin-2/Derlin-3R151.6HRD ligase componenttm5861499 bp deletion
RNF5C16C10.7RMA1 ligasetm794647 bp deletion
p97 (VCP)C06A1.1Extractortm544688 bp deletionTested
p97 (VCP)C41C4.8Extractortm659639 bp deletionTested
UFD-2T05H10.5p97 (VCP) cofactortm1380915 bp deletionTested
UFD-3C05C10.6p97 (VCP) cofactortm2915747 bp deletionTested
RAD-23ZK20.3Proteasome adaptortm3690; tm2595432 bp deletion; 677 bp deletion + 28 bp insertionTested
UBQLN-2F15C11.2aProteasome adaptortm1574755 bp deletionTested
  • aThere are ∼25 ERAD genes predicted to be conserved in C. elegans. Of these, viable mutants are available in many and were used in a candidate gene approach to identify novel modifiers of mutTDP-43 toxicity in C. elegans. Several conserved mammalian ERAD genes in the nematode have no mutants available at the time of this study, including the following: Ubc14 (Y87G2A0.9); Ube2K (F40G9.3); Ube2G (F58A4.10.1); ERManI (T03G11.4); Npl4 (F59E12.5); Ufd-1 (F19B6.2a); Ube2J1/Ube2J2 (D1022.1a); and Ube2G2 (Y87G2A0.9).