Table 1.

Components and substrates in the ERAD pathway.

Components/complexes Human (yeast)	Substrates	Function(s)	References
Substrate recognition ERMan1 (Mns1p)	ASGPR H2a	Glycan trimming	Avezov et al. (2008)
EDEM1, EDEM2, EDEM3 (Htm1p)	NHK AAT variant	Glycan trimming	Hosokawa et al. (2001), Hirao et al (2006), Hosokawa et al. (2010), Hotamisligil (2010), and Ninagawa et al. (2014)
GI/GII		Glycan trimming	D’Alessio and Dahms (2015)
OS-9 (Yos9p)	NHK AAT variant	Glycan binding	Bhamidipati et al. (2005), Szathmary et al. (2005), and Christianson et al. (2008, 2011)
XTP3-B		Glycan binding	Christianson et al. (2011) and Tyler et al. (2012)
GRP170	misfolded glycosylated client NHK	Nucleotide exchange factor	Inoue and Tsai (2016)
BiP (Kar2p)	nonsecreted Igκ light chain	Substrate recognition and recruitment	Okuda-Shimizu and Hendershot (2007)
PDI (Pdi1)	glyco-pro-α-factor; BACE457	Disulfide bonds rearrangement	Gillece et al. (1999), Molinari et al. (2002)
ERdj5-EDEM1-BiP		Substrate recognition	Ushioda et al. (2008)
UGGT		Glycoprotein glucosyltransferase	D’Alessio et al. (2010)
GRP94	NHK AAT variant	Chaperone	Christianson et al. (2008)
ERdj4	surfactant protein C	Co-chaperone	Dong et al. (2008)
ERdj5 (Scj1p)	NHK AAT variant	Co-chaperone	Dong et al. (2008) and Ushioda et al. (2008)
SEC22b (Sec22)		ER-phagosome traffic mediator	Alloatti et al. (2017)
ERp57	MHC-I heavy chain	Glycoprotein folding	Zhang, Baig et al. (2006)
Retrotranslocation and Ubiquitination Derlin-1,2,3 (Der1p)	US11-associated MHC-I heavy chain	Retrotranslocation channel	Lilley and Ploegh (2004) and Ye et al. (2004)
SEL1L-OS9-UBC6E-AUP1	US11-associated MHC-I heavy chain	Substrate recognition and dislocation	Mueller et al. (2008)
SEL1L (Hrd3p)	Igμ chain; US11-associated MHCI heavy chain	Substrate recognition and recruitment	Cattaneo et al. (2008) and Mueller et al. (2008)
SEC61 (Sec61p)		Retrotranslocation channel	Koopmann et al. (2000)
RMA1	mutant CFTR	E3 ubiquitin ligase	Morito et al. (2008)
TMEM129	US11-associated MHC-I heavy chain	E3 ubiquitin ligase	van de Weijer et al. (2014) and van den Boomen et al. (2014)
TMEM129-Derlin1	US11-associated MHC-I heavy chain	Substrate ubiquitination and translocation	van de Weijer et al. (2014) and van den Boomen et al. (2014)
HRD1-UBE2J1	non-β2m bound MHC I heavy chain	Substrate ubiquitination	Burr et al. (2011)
UBE2G2 (Ubc7p)	HMGCR	E2-conjugating enzyme	Miao et al. (2010)
SKP1-Cullin-F-box	Intergrin-β1	E3 ligase complex	Yoshida et al. (2005)
UBE2J1 (Ubc6p)	TCRα; mutant CFTR	E2-conjugating enzyme	Lenk et al. (2002) and Miao et al. (2010)
CHIP	mutant CFTR	U-box ubiquitin ligase	Younger et al. (2006)
TRC8	US2-associated MHC-I heavy chain	E3 ubiquitin ligase	Stagg et al. (2009)
HRD1 (Hrd1p)	unassembled Igμ; FAS; BLIMP-1; Nrf1	E3 ubiquitin ligase	Cattaneo et al. (2008), Tsuchiya et al. (2011), Yang et al. (2014), and Kong et al. (2016)
SEL1L-HRD1	IRE1α; P53; Pre-BCR; pro-AVP	Substrate ubiquitination and retrotranslocation	Yamasaki et al. (2007), Sun et al. (2015), Ji et al. (2016), and Shi et al. (2017)
HERP (Usa1p)	TCRα	Substrate ubiquitination	Schulze et al. (2005)
GP78	TCRα	E3 ubiquitin ligase	Zhang, Xu, et al. (2015)
TEB4 (Doa10)		E3 ubiquitin ligase	Hassink et al. (2005)
WDR20	TCRα	Adaptor Protein	Ju et al. (2018)
Dislocation and Degradation UBXD8 (Ubx2p)	ApoB100	P97 recruitment	Suzuki et al. (2012)
NGly (Png1)	glycosylated RTA	Deglycosylating enzyme	Kim et al. (2006)
RAD23 (Rad23)	glycosylated RTA	Ubiquitin binding protein	Kim et al. (2006)
AUP1 (Cue1p)	HMGCR	UBE2G2 recruitment	Jo et al. (2013)
Ataxin-3	TCRα; CD3δ	Deubiquitinating enzyme	Zhong and Pittman (2006)
P97-UFD1-NPL4 (Cdc48p-Ufd1p-Npl4p)		Substrates dislocation	Ye et al. (2001)
HERP (Usa1p)	CD3δ	Shuttle factors	Kim et al. (2008)
YOD1	TCRα; AAT	Deubiquitinating enzyme	Ernst et al. (2009)
BAG6-UBL4A-TRC35		Shuttle factors	Xu et al. (2012)
USP13	TCRα	Deubiquitinating enzyme	Sowa et al. (2009)