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| EMP46 | Protein EMP46; Integral membrane component of ER-derived COPII-coated vesicles; functions in ER to Golgi transport; EMP46 has a paralog, EMP47, that arose from the whole genome duplication. (444 aa) | ||||
| CNE1 | Calnexin; integral membrane ER chaperone involved in folding and quality control of glycoproteins; chaperone activity is inhibited by Mpd1p, with which Cne1p interacts; 24% identical to mammalian calnexin; Ca+ binding not yet shown in yeast. (502 aa) | ||||
| ALG3 | Dol-P-Man:Man(5)GlcNAc(2)-PP-Dol alpha-1,3-mannosyltransferase; Dolichol-P-Man dependent alpha(1-3) mannosyltransferase; involved in synthesis of dolichol-linked oligosaccharide donor for N-linked glycosylation of proteins; G353A missense mutation in human ortholog ALG3 implicated in carbohydrate deficient glycoprotein syndrome type IV, which is characterized by microcephaly, severe epilepsy, minimal psychomotor development, partial deficiency of sialic acids in serum glycoproteins; wild-type human ALG3 can complement yeast alg3 mutant; Belongs to the glycosyltransferase 58 family. (458 aa) | ||||
| ALG14 | UDP-N-acetylglucosamine transferase subunit ALG14; Component of UDP-GlcNAc transferase; required for second step of dolichyl-linked oligosaccharide synthesis; anchors catalytic subunit Alg13p to ER membrane; similar to bacterial and human glycosyltransferases; both human homologs ALG13 and ALG14 are required to complement yeast alg14 mutant. (237 aa) | ||||
| ALG1 | Chitobiosyldiphosphodolichol beta-mannosyltransferase; Mannosyltransferase; involved in asparagine-linked glycosylation in the endoplasmic reticulum (ER); essential for viability; human homolog ALG1 complements yeast null mutant. (449 aa) | ||||
| NPL4 | Nuclear protein localization protein 4; Substrate-recruiting cofactor of the Cdc48p-Npl4p-Ufd1p segregase; assists Cdc48p in the dislocation of misfolded, polyubiquitinated ERAD substrates that are subsequently delivered to the proteasome for degradation; also involved in the regulated destruction of resident ER membrane proteins, such as HMG-CoA reductase (Hmg1/2p) and cytoplasmic proteins (Fbp1p); role in mobilizing membrane bound transcription factors by regulated ubiquitin/proteasome-dependent processing (RUP). (580 aa) | ||||
| DER1 | Degradation in the endoplasmic reticulum protein 1; ER membrane protein that promotes export of misfolded polypeptides; required for ER-associated protein degradation of misfolded or unassembled proteins; initiates export of aberrant polypeptides from ER lumen by threading them into ER membrane and routing them to Hrd1p for ubiquitination; function normally requires N-terminal acetylation by NatB; N- and C- termini protrude into cytoplasm; similar to Dfm1p; homolog of mammalian derlin-1. (211 aa) | ||||
| ALG7 | UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; UDP-N-acetyl-glucosamine-1-P transferase; transfers Glc-Nac-P from UDP-GlcNac to Dol-P in the ER in the first step of the dolichol pathway of protein asparagine-linked glycosylation; inhibited by tunicamycin; human homolog DPAGT1 can complement yeast ALG7 mutant; Belongs to the glycosyltransferase 4 family. (448 aa) | ||||
| CDC48 | Cell division control protein 48; AAA ATPase; subunit of polyUb-selective segregase complex involved in ERAD, INM-associated degradation (INMAD), mitotic spindle disassembly, macroautophagy, PMN, ribosome-associated degradation, ribophagy, homotypic ER membrane fusion, SCF complex disassembly, cell wall integrity during heat stress, and telomerase regulation; mobilizes membrane-anchored transcription factors by regulated Ub/proteasome-dependent processing (RUP); human ortholog VCP complements a cdc48 mutant. (835 aa) | ||||
| YOS9 | Protein OS-9 homolog; ER quality-control lectin; integral subunit of the HRD ligase; participates in efficient ER retention of misfolded proteins by recognizing them and delivering them to Hrd1p; binds to glycans with terminal alpha-1,6 linked mannose on misfolded N-glycosylated proteins and participates in targeting proteins to ERAD; member of the OS-9 protein family. (542 aa) | ||||
| GTB1 | Glucosidase II beta subunit, forms a complex with alpha subunit Rot2p; involved in removal of two glucose residues from N-linked glycans during glycoprotein biogenesis in the ER; relocalizes from ER to cytoplasm upon DNA replication stress. (702 aa) | ||||
| MNN1 | Alpha-1,3-mannosyltransferase; integral membrane glycoprotein of the Golgi complex, required for addition of alpha1,3-mannose linkages to N-linked and O-linked oligosaccharides, one of five S. cerevisiae proteins of the MNN1 family; Belongs to the MNN1/MNT family. (762 aa) | ||||
| EMP47 | Protein EMP47; Integral membrane component of ER-derived COPII-coated vesicles; functionS in ER to Golgi transport; EMP47 has a paralog, EMP46, that arose from the whole genome duplication; Belongs to the EMP46/EMP47 family. (445 aa) | ||||
| CWH41 | Processing alpha glucosidase I; ER type II integral membrane N-glycoprotein involved in assembly of cell wall beta 1,6 glucan and asparagine-linked protein glycosylation; also involved in ER protein quality control and sensing of ER stress; Belongs to the glycosyl hydrolase 63 family. (833 aa) | ||||
| OCH1 | Initiation-specific alpha-1,6-mannosyltransferase; Mannosyltransferase of the cis-Golgi apparatus; initiates the polymannose outer chain elongation of N-linked oligosaccharides of glycoproteins; Belongs to the glycosyltransferase 32 family. (480 aa) | ||||
| ALG13 | UDP-N-acetylglucosamine transferase subunit ALG13; Catalytic component of UDP-GlcNAc transferase; required for the second step of dolichyl-linked oligosaccharide synthesis; anchored to the ER membrane via interaction with Alg14p; similar to bacterial and human glycosyltransferases; protein abundance increases in response to DNA replication stress; both human homologs ALG13 and ALG14 are required to complement yeast alg13 mutant. (202 aa) | ||||
| UFD1 | Ubiquitin fusion degradation protein 1; Substrate-recruiting cofactor of the Cdc48p-Npl4p-Ufd1p segregase; polyubiquitin binding protein that assists in the dislocation of misfolded, ERAD substrates that are subsequently delivered to the proteasome for degradation; involved in regulated destruction of ER membrane proteins such as HMG-CoA reductase (Hmg1/2p) and cytoplasmic proteins (Fbp1p); involved in mobilizing membrane bound transcription factors by regulated Ub/proteasome-dependent processing (RUP). (361 aa) | ||||
| MNL1 | ER degradation-enhancing alpha-mannosidase-like protein 1; Alpha-1,2-specific exomannosidase of the endoplasmic reticulum; involved in glycan trimming of both folded and misfolded glycoproteins; complexes with Pdi1p, and trims a mannose from Man8GlcNac2 glycans to generate Man7GlcNac2, an oligosaccharide signal on glycoproteins destined for ER-associated protein degradation; requires Pdi1p for stability and substrate recognition; human homolog EDEM1 can complement yeast null mutant; Belongs to the glycosyl hydrolase 47 family. (796 aa) | ||||
| MNN4 | Protein MNN4; Putative positive regulator of mannosylphosphate transferase Mnn6p; involved in mannosylphosphorylation of N-linked oligosaccharides; expression increases in late-logarithmic and stationary growth phases; coding sequence contains length polymorphisms in different strains; MNN4 has a paralog, YJR061W, that arose from the whole genome duplication; To yeast YJR061w. (1178 aa) | ||||
| HRD3 | ERAD-associated E3 ubiquitin-protein ligase component HRD3; ER membrane protein that plays a central role in ERAD; forms HRD complex with Hrd1p and ER-associated protein degradation (ERAD) determinants that engages in lumen to cytosol communication and coordination of ERAD events; Belongs to the sel-1 family. (833 aa) | ||||
| UBX2 | UBX domain-containing protein 2; Bridging factor involved in ER-associated protein degradation (ERAD); bridges the cytosolic Cdc48p-Npl1p-Ufd1p ATPase complex and the membrane associated Ssm4p and Hrd1p ubiquitin ligase complexes; contains a UBX (ubiquitin regulatory X) domain and a ubiquitin-associated (UBA) domain; redistributes from the ER to lipid droplets during the diauxic shift and stationary phase; required for the maintenance of lipid homeostasis. (584 aa) | ||||
| USA1 | U1 SNP1-associating protein 1; Scaffold subunit of the Hrd1p ubiquitin ligase; also promotes ligase oligomerization; involved in ER-associated protein degradation (ERAD); interacts with the U1 snRNP-specific protein, Snp1p. (838 aa) | ||||
| ALG11 | Alpha-1,2-mannosyltransferase; catalyzes sequential addition of the two terminal alpha 1,2-mannose residues to the Man5GlcNAc2-PP-dolichol intermediate during asparagine-linked glycosylation in the ER. (548 aa) | ||||
| ALG12 | Alpha-1,6-mannosyltransferase localized to the ER; responsible for addition of alpha-1,6 mannose to dolichol-linked Man7GlcNAc2; acts in the dolichol pathway for N-glycosylation; human homolog ALG12 complements yeast null mutant. (551 aa) | ||||
| HRD1 | ERAD-associated E3 ubiquitin-protein ligase HRD1; Ubiquitin-protein ligase involved in ER-associated degradation (ERAD) of misfolded proteins; upon autoubiquitination triggers retrotranslocation of misfolded proteins to cytosol for degradation; genetically linked to the unfolded protein response (UPR); regulated through association with Hrd3p; contains an H2 ring finger; likely plays a general role in targeting proteins that persistently associate with and potentially obstruct the ER-localized translocon; Belongs to the HRD1 family. (551 aa) | ||||
| ALG6 | Alpha 1,3 glucosyltransferase; involved in transfer of oligosaccharides from dolichyl pyrophosphate to asparagine residues of proteins during N-linked protein glycosylation; C998T transition in human ortholog ALG6 causes carbohydrate-deficient glycoprotein syndrome type-Ic; wild-type human ortholog ALG6 can partially complement yeast alg6 mutant; Belongs to the ALG6/ALG8 glucosyltransferase family. (544 aa) | ||||
| ALG8 | Dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase; Glucosyl transferase; involved in N-linked glycosylation; adds glucose to the dolichol-linked oligosaccharide precursor prior to transfer to protein during lipid-linked oligosaccharide biosynthesis; similar to Alg6p; human homolog ALG8 can complement yeast null mutant; Belongs to the ALG6/ALG8 glucosyltransferase family. (577 aa) | ||||
| KRE5 | Killer toxin-resistance protein 5; Protein required for beta-1,6 glucan biosynthesis; mutations result in aberrant morphology and severe growth defects. (1365 aa) | ||||
| PNG1 | Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase; Conserved peptide N-glycanase; deglycosylating enzyme that cleaves N-glycans that are attached to misfolded ERAD substrate glycoproteins prior to proteasome-dependent degradation; localizes to the cytoplasm and nucleus; activity is enhanced by interaction with Rad23p; human ortholog NGLY1 is associated with a syndrome characterized by developmental delays, epilepsy, absence of tears and liver disease; Belongs to the transglutaminase-like superfamily. PNGase family. (363 aa) | ||||