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| 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) | ||||
| VPS4 | AAA-ATPase involved in multivesicular body (MVB) protein sorting; ATP-bound Vps4p localizes to endosomes and catalyzes ESCRT-III disassembly and membrane release; ATPase activity is activated by Vta1p; regulates cellular sterol metabolism. (437 aa) | ||||
| ATG5 | Autophagy protein 5; Conserved protein involved in autophagy and the Cvt pathway; undergoes conjugation with Atg12p to form a complex involved in Atg8p lipidation; Atg5p-Atg12p conjugate enhances E2 activity of Atg3 by rearranging its catalytic site, also forms a complex with Atg16p; the Atg5-Atg12/Atg16 complex binds to membranes and is essential for autophagosome formation; also involved in methionine restriction extension of chronological lifespan in an autophagy-dependent manner. (294 aa) | ||||
| SSE1 | ATPase component of heat shock protein Hsp90 chaperone complex; serves as nucleotide exchange factor to load ATP onto the SSA class of cytosolic Hsp70s; plays a role in prion propagation and determining prion variants; binds unfolded proteins; member of Hsp110 subclass of HSP70 proteins; deletion results in spindle elongation in S phase; SSE1 has a paralog, SSE2, that arose from the whole genome duplication. (693 aa) | ||||
| ATG40 | Autophagy-related protein 40; Autophagy receptor with a role in endoplasmic reticulum degradation; involved specifically in autophagy of cortical and cytoplasmic ER in response to nitrogen starvation or rapamycin treatment; localizes to the cortical and cytoplasmic ER; similar to human FAM134B, which is also involved in ER autophagy and is associated with sensory neuropathy. (256 aa) | ||||
| URE2 | Nitrogen catabolite repression transcriptional regulator; inhibits GLN3 transcription in good nitrogen source; role in sequestering Gln3p and Gat1p to the cytoplasm; has glutathione peroxidase activity and can mutate to acquire GST activity; self-assembly under limited nitrogen conditions creates [URE3] prion and releases catabolite repression. (354 aa) | ||||
| ASI2 | Protein ASI2; Subunit of the inner nuclear membrane Asi ubiquitin ligase complex; the Asi complex targets both misfolded proteins of the inner nuclear membrane-associated degradation (INMAD) pathway and inner for ubiquitin-mediated degradation; acts with Asi1p and Asi3p to ensure the fidelity of SPS-sensor signaling by targeting latent unprocessed forms of Stp1p and Stp2p, maintaining the repressed state of gene expression in the absence of inducing amino acids. (289 aa) | ||||
| YDJ1 | Mitochondrial protein import protein MAS5; Type I HSP40 co-chaperone; involved in regulation of HSP90 and HSP70 functions; acts as an adaptor that helps Rsp5p recognize cytosolic misfolded proteins for ubiquitination after heat shock; critical for determining cell size at Start as a function of growth rate; involved in protein translocation across membranes; member of the DnaJ family; chimeric protein in which human p58IPK J domain replaces yeast Ydj1p J domain can complement yeast ydj1 mutant. (409 aa) | ||||
| ASI3 | Protein ASI3; Subunit of the inner nuclear membrane Asi ubiquitin ligase complex; Asi complex targets both misfolded proteins of the inner nuclear membrane-associated degradation (INMAD) pathway and regulators of sterol biosynthesis for ubiquitin-mediated degradation; acts with Asi1p and Asi2p to ensure the fidelity of SPS-sensor signaling by targeting latent unprocessed forms of Stp1p and Stp2p, maintaining the repressed state of gene expression in the absence of inducing amino acids. (676 aa) | ||||
| SIS1 | Protein SIS1; Type II HSP40 co-chaperone that interacts with the HSP70 protein Ssa1p; shuttles between cytosol and nucleus; mediates delivery of misfolded proteins into the nucleus for degradation; involved in proteasomal degradation of misfolded cytosolic proteins; protein abundance increases in response to DNA replication stress; polyQ aggregates sequester Sis1p and interfere with clearance of misfolded proteins; similar to bacterial DnaJ proteins and mammalian DnaJB1. (352 aa) | ||||
| PRC1 | Vacuolar carboxypeptidase Y (proteinase C, CPY); broad-specificity C-terminal exopeptidase involved in non-specific protein degradation in the vacuole; member of the serine carboxypeptidase family. (532 aa) | ||||
| DSK2 | Nuclear-enriched ubiquitin-like polyubiquitin-binding protein; required for spindle pole body (SPB) duplication and for transit through the G2/M phase of the cell cycle; involved in proteolysis; interacts with the proteasome; protein abundance increases in response to DNA replication stress. (373 aa) | ||||
| CUE1 | Coupling of ubiquitin conjugation to ER degradation protein 1; Ubiquitin-binding protein; ER membrane protein that recruits and integrates the ubiquitin-conjugating enzyme Ubc7p into ER membrane-bound ubiquitin ligase complexes that function in the ER-associated degradation (ERAD) pathway for misfolded proteins; contains a CUE domain that binds ubiquitin to facilitate intramolecular monoubiquitination and to promote diubiquitin elongation, facilitating polyubiquitin chain formation. (203 aa) | ||||
| RKR1 | RING domain E3 ubiquitin ligase; involved in ubiquitin-mediated degradation of non-stop proteins and translationally stalled ER membrane proteins; component of ribosome-bound RQC (ribosome quality control) complex; degrades products of mRNAs lacking a termination codon regardless of a poly(A) tail; functional connections to chromatin modification; homolog of mouse Listerin, mutations in which reported to cause neurodegeneration; Belongs to the LTN1 family. (1562 aa) | ||||
| SCJ1 | DnaJ-related protein SCJ1; One of several homologs of bacterial chaperone DnaJ; located in the ER lumen where it cooperates with Kar2p to mediate maturation of proteins. (377 aa) | ||||
| ATG16 | Conserved protein involved in autophagy; interacts with Atg12p-Atg5p conjugates to form Atg12p-Atg5p-Atg16p multimers, which binds to membranes and localizes to the pre-autophagosomal structure and are required for autophagy; relocalizes from nucleus to cytoplasmic foci upon DNA replication stress. (150 aa) | ||||
| ASI1 | Protein ASI1; Subunit of the inner nuclear membrane Asi ubiquitin ligase complex; the Asi complex targets both misfolded proteins of the inner nuclear membrane-associated degradation (INMAD) pathway and regulators of sterol biosynthesis for ubiquitin-mediated degradation; acts with Asi2p and Asi3p to ensure the fidelity of SPS-sensor signaling by targeting latent unprocessed forms of Stp1p and Stp2p, maintaining the repressed state of gene expression in the absence of inducing amino acids. (624 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) | ||||
| 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) | ||||
| HMG2 | HMG-CoA reductase; converts HMG-CoA to mevalonate, a rate-limiting step in sterol biosynthesis; one of two isozymes; overproduction induces assembly of peripheral ER membrane arrays and short nuclear-associated membrane stacks; forms foci at nuclear periphery upon DNA replication stress; HMG2 has a paralog, HMG1, that arose from the whole genome duplication; human homolog HMGCR can complement yeast hmg2 mutant. (1045 aa) | ||||
| SEC61 | Conserved ER protein translocation channel; essential subunit of Sec61 complex (Sec61p, Sbh1p, and Sss1p); forms channel for SRP-dependent protein import; with Sec63 complex allows SRP-independent protein import into ER; involved in posttranslational soluble protein import into the ER, ERAD of soluble substrates, and misfolded soluble protein export from the ER. (480 aa) | ||||
| ATG39 | Autophagy-related protein 39; Autophagy receptor with a role in degradation of the ER and nucleus; involved specifically in autophagy of perinuclear endoplasmic reticulum in response to nitrogen starvation or rapamycin treatment; localizes to the perinuclear ER. (398 aa) | ||||
| UBI4 | Polyubiquitin; Ubiquitin; becomes conjugated to proteins, marking them for selective degradation via the ubiquitin-26S proteasome system; essential for the cellular stress response; encoded as a polyubiquitin precursor comprised of 5 head-to-tail repeats; protein abundance increases in response to DNA replication stress. (381 aa) | ||||
| STE6 | Alpha-factor-transporting ATPase; Plasma membrane ATP-binding cassette (ABC) transporter; required for the export of a-factor, catalyzes ATP hydrolysis coupled to a-factor transport; contains 12 transmembrane domains and two ATP binding domains; expressed only in MATa cells; human homolog ABCB1 mediates multidrug resistance in many chemotherapy-resistant tumors by effluxing toxic compounds from the cell. (1290 aa) | ||||
| YPF1 | Intramembrane aspartyl protease of the perinuclear ER membrane; acts in a branch of ER-associated degradation (ERAD) that degrades functional proteins rather than misfolded proteins; regulates abundance of high-affinity plasma membrane transporters, such as Ctr1p and Zrt1p, during the starvation response; has a presenilin fold; member of the GxGD family of intramembrane proteases; closest human homolog is signal peptide peptidase (SPP). (587 aa) | ||||
| STE24 | CAAX prenyl protease 1; Highly conserved zinc metalloprotease; functions in two steps of a-factor maturation, C-terminal CAAX proteolysis and the first step of N-terminal proteolytic processing; cleaves both isoprenylated and non-prenylated oligopeptides; contains multiple transmembrane spans; human homolog ZMPSTE24 implicated in mandibuloacral dysplasia (MAD), and can complement yeast null mutant. (453 aa) | ||||
| NUP85 | Nucleoporin NUP85; Subunit of the Nup84p subcomplex of the nuclear pore complex (NPC); contributes to nucleocytoplasmic transport and NPC biogenesis and is involved in establishment of a normal nucleocytoplasmic concentration gradient of the GTPase Gsp1p; also plays roles in several processes that may require localization of genes or chromosomes at the nuclear periphery, including double-strand break repair, transcription and chromatin silencing; homologous to human NUP85 aka NUP75. (744 aa) | ||||
| KAR2 | Endoplasmic reticulum chaperone BiP; ATPase involved in protein import into the ER; also acts as a chaperone to mediate protein folding in the ER and may play a role in ER export of soluble proteins; regulates the unfolded protein response via interaction with Ire1p. (682 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) | ||||
| ERG11 | Lanosterol 14-alpha-demethylase; catalyzes C-14 demethylation of lanosterol to form 4,4''-dimethyl cholesta-8,14,24-triene-3-beta-ol in ergosterol biosynthesis pathway; transcriptionally down-regulated when ergosterol is in excess; member of cytochrome P450 family; associated and coordinately regulated with the P450 reductase Ncp1p; human CYP51A1 functionally complements the lethality of the erg11 null mutation. (530 aa) | ||||
| UBR1 | E3 ubiquitin ligase (N-recognin); heterodimerizes with Rad6p to recognize and ubiquitinate substrates of the N-end rule pathway; role in endoplasmic reticulum-associated protein degradation (ERAD) in the absence of canonical ER membrane ligases or after stress; major role in targeting misfolded cytosolic proteins for degradation; regulates peptide transport via Cup9p ubiquitination; mutation in human UBR1 causes Johansson-Blizzard Syndrome (JBS). (1950 aa) | ||||
| ERG1 | Squalene epoxidase; catalyzes the epoxidation of squalene to 2,3-oxidosqualene; plays an essential role in the ergosterol-biosynthesis pathway and is the specific target of the antifungal drug terbinafine; human SQLE functionally complements the lethality of the erg1 null mutation. (496 aa) | ||||
| CBF2 | Essential kinetochore protein; component of the CBF3 multisubunit complex that binds to the CDEIII region of the centromere; Cbf2p also binds to the CDEII region possibly forming a different multimeric complex, ubiquitinated in vivo; sumoylated in an Mms21p-dependent manner; relative distribution to the spindle pole body decreases upon DNA replication stress. (956 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) | ||||
| ZRT1 | Zinc-regulated transporter 1; High-affinity zinc transporter of the plasma membrane; responsible for the majority of zinc uptake; transcription is induced under low-zinc conditions by the Zap1p transcription factor. (376 aa) | ||||
| DEG1 | tRNA:pseudouridine synthase; introduces pseudouridines at position 38 or 39 in tRNA; also responsible for pseudouracil modification of some mRNAs; important for maintenance of translation efficiency and normal cell growth, localizes to both the nucleus and cytoplasm; non-essential for viability. (442 aa) | ||||
| SBH2 | Ssh1p-Sss1p-Sbh2p complex component; involved in protein translocation into the endoplasmic reticulum; SBH2 has a paralog, SBH1, that arose from the whole genome duplication; Belongs to the SEC61-beta family. (88 aa) | ||||
| RAD23 | UV excision repair protein RAD23; Protein with ubiquitin-like N terminus; subunit of Nuclear Excision Repair Factor 2 (NEF2) with Rad4p that binds damaged DNA; enhances protein deglycosylation activity of Png1p; also involved, with Rad4p, in ubiquitylated protein turnover; Rad4p-Rad23p heterodimer binds to promoters of DNA damage response genes to repress their transcription in the absence of DNA damage. (398 aa) | ||||
| STP1 | Transcription factor; contains a N-terminal regulatory motif (RI) that acts as a cytoplasmic retention determinant and as an Asi dependent degron in the nucleus; undergoes proteolytic processing by SPS (Ssy1p-Ptr3p-Ssy5p)-sensor component Ssy5p in response to extracellular amino acids; activates transcription of amino acid permease genes and may have a role in tRNA processing; STP1 has a paralog, STP2, that arose from the whole genome duplication. (519 aa) | ||||
| DFM1 | DER1-like family member protein 1; Endoplasmic reticulum (ER) localized protein; involved in ER-associated protein degradation (ERAD), ER stress, and homeostasis; interacts with components of ERAD-L and ERAD-C and Cdc48p; derlin-like family member similar to Der1p; Belongs to the derlin family. (341 aa) | ||||
| PMT2 | Dolichyl-phosphate-mannose--protein mannosyltransferase 2; Protein O-mannosyltransferase of the ER membrane; transfers mannose residues from dolichyl phosphate-D-mannose to protein serine/threonine residues; involved in ER quality control; acts in a complex with Pmt1p, can instead interact with Pmt5p; antifungal drug target; PMT2 has a paralog, PMT3, that arose from the whole genome duplication. (759 aa) | ||||
| ATG8 | Autophagy-related protein 8; Component of autophagosomes and Cvt vesicles; regulator of Atg1p, targets it to autophagosomes; binds the Atg1p-Atg13p complex, triggering its vacuolar degradation; unique ubiquitin-like protein whose conjugation target is lipid phosphatidylethanolamine (PE); Atg8p-PE is anchored to membranes, is involved in phagophore expansion, and may mediate membrane fusion during autophagosome formation; deconjugation of Atg8p-PE is required for efficient autophagosome biogenesis. (117 aa) | ||||
| UBC4 | Ubiquitin-conjugating enzyme (E2); key E2 partner with Ubc1p for the anaphase-promoting complex (APC); mediates degradation of abnormal or excess proteins, including calmodulin and histone H3; regulates levels of DNA Polymerase-{alpha} to promote efficient and accurate DNA replication; interacts with many SCF ubiquitin protein ligases; component of the cellular stress response; UBC4 has a paralog, UBC5, that arose from the whole genome duplication. (148 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) | ||||
| ATG12 | Ubiquitin-like protein ATG12; Ubiquitin-like modifier involved in autophagy and the Cvt pathway; conserved; conjugated to Atg5p to form a complex involved in Atg8p lipidation; Atg5p-Atg12p conjugate enhances E2 activity of Atg3p by rearranging its catalytic site, also forms a complex with Atg16p; the Atg5-Atg12/Atg16 complex binds to membranes and is essential for autophagosome formation. (186 aa) | ||||
| PDI1 | Protein disulfide isomerase; multifunctional oxidoreductase of the ER lumen, essential for disulfide bond formation in secretory and cell-surface proteins, processing of non-native disulfide bonds; Ero1p activator; complexes with exomannosidase, Mnl1p to facilitate the recognition of misfolded glycoproteins and the trimming of glycan Man8GlcNAc2 to Man7GlcNAc2 on substrates, thereby accelerating ERAD; PDI1 has a paralog, EUG1, that arose from the whole genome duplication. (522 aa) | ||||
| HMLALPHA2 | Silenced copy of ALPHA2 at HML; homeobox-domain protein that associates with Mcm1p in haploid cells to repress a-specific gene expression and interacts with a1p in diploid cells to repress haploid-specific gene expression. (210 aa) | ||||
| MATALPHA2 | Homeobox-domain protein; with Mcm1p, represses a-specific genes in haploids; acts with A1p to repress transcription of haploid-specific genes in diploids; one of two genes encoded by the MATalpha mating type cassette. (210 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) | ||||
| UFD2 | E4 ubiquitin-protein ligase UFD2; Ubiquitin chain assembly factor (E4); cooperates with a ubiquitin-activating enzyme (E1), a ubiquitin-conjugating enzyme (E2), and a ubiquitin protein ligase (E3) to conjugate ubiquitin to substrates; also functions as an E3. (961 aa) | ||||
| UBC1 | Ubiquitin-conjugating enzyme; key E2 partner with Ubc4p for the anaphase-promoting complex (APC); mediates selective degradation of short-lived and abnormal proteins; plays a role in vesicle biogenesis and ER-associated protein degradation (ERAD); component of the cellular stress response; protein abundance increases in response to DNA replication stress key E2 partner with Ubc4p for the anaphase-promoting complex (APC). (215 aa) | ||||