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.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

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.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

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.

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.

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.

Heat shock protein 70 homolog LHS1; Molecular chaperone of the endoplasmic reticulum lumen; involved in polypeptide translocation and folding; nucleotide exchange factor for the ER lumenal Hsp70 chaperone Kar2p; regulated by the unfolded protein response pathway.

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.

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.

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.

Protein translocation protein SEC63; Essential subunit of Sec63 complex; with Sec61 complex, Kar2p/BiP and Lhs1p forms a channel competent for SRP-dependent and post-translational SRP-independent protein targeting and import into the ER; other members are Sec62p, Sec66p, and Sec72p.

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.

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.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

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.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

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.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

Beta-isopropylmalate dehydrogenase (IMDH); catalyzes the third step in the leucine biosynthesis pathway; can additionally catalyze the conversion of beta-ethylmalate into alpha-ketovalerate; Belongs to the isocitrate and isopropylmalate dehydrogenases family.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

Heat shock protein 70 homolog LHS1; Molecular chaperone of the endoplasmic reticulum lumen; involved in polypeptide translocation and folding; nucleotide exchange factor for the ER lumenal Hsp70 chaperone Kar2p; regulated by the unfolded protein response pathway.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

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.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

Protein translocation protein SEC63; Essential subunit of Sec63 complex; with Sec61 complex, Kar2p/BiP and Lhs1p forms a channel competent for SRP-dependent and post-translational SRP-independent protein targeting and import into the ER; other members are Sec62p, Sec66p, and Sec72p.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

tRNA ligase; required for tRNA splicing and for both splicing and translation of HAC1 mRNA in the UPR; has phosphodiesterase, polynucleotide kinase, and ligase activities; localized at the inner nuclear envelope and partially to polysomes.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

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.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

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.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

Endoplasmic oxidoreductin-1; Thiol oxidase required for oxidative protein folding in the ER; essential for maintaining ER redox balance; feedback regulated via reduction and oxidation of regulatory bonds; reduced Pdi1p activates Ero1p by direct reduction of Ero1p regulatory bonds; depletion of thiol substrates and accumulation of oxidized Pdi1p results in inactivation of Ero1p by both Pdi1p-mediated oxidation and autonomous oxidation of Ero1p regulatory bonds; ero1-1 mutation complemented by human ERO1L.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

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.

Transcriptional activator HAC1; Basic leucine zipper (bZIP) transcription factor (ATF/CREB1 homolog); regulates the unfolded protein response, via UPRE binding, and membrane biogenesis; ER stress-induced splicing pathway facilitates efficient Hac1p synthesis; two functional forms of Hac1p are produced; translation initiation is repressed under non-stress conditions; protein abundance increases in response to DNA replication stress.

Beta-isopropylmalate dehydrogenase (IMDH); catalyzes the third step in the leucine biosynthesis pathway; can additionally catalyze the conversion of beta-ethylmalate into alpha-ketovalerate; Belongs to the isocitrate and isopropylmalate dehydrogenases family.