PATHWAY: ko04141 Help
Entry Name Protein processing in endoplasmic reticulum
Description The endoplasmic reticulum (ER) is a subcellular organelle where proteins are folded with the help of lumenal chaperones. Newly synthesized peptides enter the ER via the sec61 pore and are glycosylated. Correctly folded proteins are packaged into transport vesicles that shuttle them to the Golgi complex. Misfolded proteins are retained within the ER lumen in complex with molecular chaperones. Proteins that are terminally misfolded bind to BiP and are directed toward degradation through the proteasome in a process called ER-associated degradation (ERAD). Accumulation of misfolded proteins in the ER causes ER stress and activates a signaling pathway called the unfolded protein response (UPR). In certain severe situations, however, the protective mechanisms activated by the UPR are not sufficient to restore normal ER function and cells die by apoptosis.
Class Genetic Information Processing; Folding, sorting and degradationBRITE hierarchy
Pathway map ko04141 Protein processing in endoplasmic reticulum
Other DBs Orthology K00730 OST4; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit 4
K01230 MAN1A_C, MNS1_2; mannosyl-oligosaccharide alpha-1,2-mannosidase [EC:3.2.1.113 ]
K01456 E3.5.1.52, NGLY1, PNG1; peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase [EC:3.5.1.52 ]
K02159 BAX; apoptosis regulator BAX
K02161 BCL2; apoptosis regulator Bcl-2
K03094 SKP1, CBF3D; S-phase kinase-associated protein 1
K03237 EIF2S1; translation initiation factor 2 subunit 1
K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
K04079 HSP90A, htpG; molecular chaperone HtpG
K04374 ATF4, CREB2; cyclic AMP-dependent transcription factor ATF-4
K04426 MAP3K5, ASK1; mitogen-activated protein kinase kinase kinase 5 [EC:2.7.11.25 ]
K04431 MAP2K7, MKK7; mitogen-activated protein kinase kinase 7 [EC:2.7.12.2 ]
K04440 JNK; mitogen-activated protein kinase 8/9/10 (c-Jun N-terminal kinase) [EC:2.7.11.24 ]
K04452 DDIT3, GADD153; DNA damage-inducible transcript 3
K04554 UBE2J2, NCUBE2, UBC6; ubiquitin-conjugating enzyme E2 J2 [EC:2.3.2.23 ]
K04571 SSR4; translocon-associated protein subunit delta
K04741 CASP12; caspase 12 [EC:3.4.22.-]
K05546 GANAB; mannosyl-oligosaccharide alpha-1,3-glucosidase [EC:3.2.1.207 ]
K05638 NFE2L2, NRF2; nuclear factor erythroid 2-related factor 2
K06689 UBE2D, UBC4, UBC5; ubiquitin-conjugating enzyme E2 D [EC:2.3.2.23 ]
K07151 STT3; dolichyl-diphosphooligosaccharide---protein glycosyltransferase [EC:2.4.99.18 ]
K07342 SEC61G, SSS1, secE; protein transport protein SEC61 subunit gamma and related proteins
K07953 SAR1; GTP-binding protein SAR1 [EC:3.6.5.-]
K08288 PRKCSH; protein kinase C substrate 80K-H
K08653 MBTPS1; membrane-bound transcription factor site-1 protease [EC:3.4.21.112 ]
K08852 ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [EC:2.7.11.1 3.1.26.-]
K08860 EIF2AK3, PERK; eukaryotic translation initiation factor 2-alpha kinase 3 [EC:2.7.11.1 ]
K09027 XBP1; X box-binding protein 1
K09049 ATF6B, CREBL1; cyclic AMP-dependent transcription factor ATF-6 beta
K09054 ATF6A; cyclic AMP-dependent transcription factor ATF-6 alpha
K09481 SEC61B, SBH2; protein transport protein SEC61 subunit beta
K09485 HSP110; heat shock protein 110kDa
K09486 HYOU1; hypoxia up-regulated 1
K09487 HSP90B, TRA1; heat shock protein 90kDa beta
K09502 DNAJA1; DnaJ homolog subfamily A member 1
K09503 DNAJA2; DnaJ homolog subfamily A member 2
K09507 DNAJB1; DnaJ homolog subfamily B member 1
K09508 DNAJB2; DnaJ homolog subfamily B member 2
K09517 DNAJB11; DnaJ homolog subfamily B member 11
K09518 DNAJB12; DnaJ homolog subfamily B member 12
K09521 DNAJC1; DnaJ homolog subfamily C member 1
K09523 DNAJC3; DnaJ homolog subfamily C member 3
K09525 DNAJC5; DnaJ homolog subfamily C member 5
K09530 DNAJC10; DnaJ homolog subfamily C member 10 [EC:1.8.4.-]
K09540 SEC63, DNAJC23; translocation protein SEC63
K09541 CRYAA; crystallin, alpha A
K09542 CRYAB; crystallin, alpha B
K09555 BAG1; BCL2-associated athanogene 1
K09556 BAG2; BCL2-associated athanogene 2
K09561 STUB1, CHIP; STIP1 homology and U-box containing protein 1 [EC:2.3.2.27 ]
K09562 HSPBP1, FES1; hsp70-interacting protein
K09584 PDIA6, TXNDC7; protein disulfide-isomerase A6 [EC:5.3.4.1 ]
K09586 ERP29; endoplasmic reticulum protein 29
K10080 LMAN1, ERGIC53; lectin, mannose-binding 1
K10081 LMAN1L, ERGL; lectin, mannose-binding 1-like
K10082 LMAN2, VIP36; lectin, mannose-binding 2
K10084 EDEM1; ER degradation enhancer, mannosidase alpha-like 1
K10085 EDEM2; ER degradation enhancer, mannosidase alpha-like 2
K10086 EDEM3; ER degradation enhancer, mannosidase alpha-like 3
K10099 FBXO2, NFB42; F-box protein 2
K10578 UBE2J1, NCUBE1, UBC6; ubiquitin-conjugating enzyme E2 J1 [EC:2.3.2.23 ]
K10601 SYVN1, HRD1; E3 ubiquitin-protein ligase synoviolin [EC:2.3.2.27 ]
K10661 MARCH6, DOA10; E3 ubiquitin-protein ligase MARCH6 [EC:2.3.2.27 ]
K10839 RAD23, HR23; UV excision repair protein RAD23
K10950 ERO1L; ERO1-like protein alpha [EC:1.8.4.-]
K10956 SEC61A; protein transport protein SEC61 subunit alpha
K10976 ERO1LB; ERO1-like protein beta [EC:1.8.4.-]
K11718 HUGT; UDP-glucose:glycoprotein glucosyltransferase [EC:2.4.1.-]
K11863 ATXN3, MJD; Ataxin-3 [EC:3.4.22.-]
K12275 SEC62; translocation protein SEC62
K12666 RPN1, OST1; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit 1 (ribophorin I)
K12667 RPN2, SWP1; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit 2 (ribophorin II)
K12668 DAD1, OST2; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit DAD1/OST2
K12669 OST3_6; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit 3/6
K12670 DDOST, WBP1; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit DDOST/WBP1
K12691 OST5; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit 5
K13249 SSR1; translocon-associated protein subunit alpha
K13250 SSR2; translocon-associated protein subunit beta
K13251 SSR3; translocon-associated protein subunit gamma
K13525 VCP, CDC48; transitional endoplasmic reticulum ATPase
K13719 OTU1, YOD1; ubiquitin thioesterase OTU1 [EC:3.1.2.-]
K13984 TXNDC5, ERP46; thioredoxin domain-containing protein 5 [EC:5.3.4.1 ]
K13993 HSP20; HSP20 family protein
K13999 CKAP4, CLIMP63; cytoskeleton-associated protein 4
K14000 RRBP1; ribosome-binding protein 1
K14001 SIL1, SLS1; nucleotide exchange factor SIL1
K14002 SCJ1; DnaJ-related protein SCJ1
K14003 PREB, SEC12; prolactin regulatory element-binding protein
K14004 SEC13; protein transport protein SEC13
K14005 SEC31; protein transport protein SEC31
K14006 SEC23; protein transport protein SEC23
K14007 SEC24; protein transport protein SEC24
K14008 ERLEC1, XTP3B; endoplasmic reticulum lectin 1
K14009 BCAP31, BAP31; B-cell receptor-associated protein 31
K14010 TRAM1; translocating chain-associated membrane protein 1
K14011 UBXN6, UBXD1; UBX domain-containing protein 6
K14012 NSFL1C, UBX1, SHP1; UBX domain-containing protein 1
K14013 UBX2, SEL1; UBX domain-containing protein 2
K14014 SVIP; small VCP/p97-interacting protein
K14015 NPLOC4, NPL4; nuclear protein localization protein 4
K14016 UFD1; ubiquitin fusion degradation protein 1
K14017 SNL1; HSP70 co-chaperone SNL1
K14018 PLAA, DOA1, UFD3; phospholipase A-2-activating protein
K14019 PPP1R15A, GADD34; protein phosphatase 1 regulatory subunit 15A
K14021 BAK, BAK1; Bcl-2 homologous antagonist/killer
K14022 CUE1, KIS4; coupling of ubiquitin conjugation to ER degradation protein 1
K14023 HRD3; ERAD-associated E3 ubiquitin-protein ligase component HRD3
K14024 USA1; U1 SNP1-associating protein 1
K14025 SELS, VIMP; selenoprotein S
K14026 SEL1, SEL1L; SEL1 protein
K14027 HERPUD1, HERP; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein
K19478 MAGT1, TUSC3; dolichyl-diphosphooligosaccharide---protein glycosyltransferase subunit MAGT1/TUSC3
K23741 MAN1B, MNS3; endoplasmic reticulum Man9GlcNAc2 1,2-alpha-mannosidase
K24348 UBXN1_4; UBX domain-containing protein 1/4
K24349 UBXN2A; UBX domain-containing protein 2A
K24351 UBXN8; UBX domain-containing protein 8
K26501 OSTC; oligosaccharyltransferase complex subunit OSTC
K26502 TMEM258; transmembrane protein 258
K28202 KRTCAP2, KCP2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit KCP2
Compound Reference Authors Naidoo N
Title ER and aging-Protein folding and the ER stress response.
Journal Reference Authors Malhotra JD, Kaufman RJ
Title The endoplasmic reticulum and the unfolded protein response.
Journal Reference Authors Maattanen P, Gehring K, Bergeron JJ, Thomas DY
Title Protein quality control in the ER: the recognition of misfolded proteins.
Journal Reference Authors Stolz A, Wolf DH
Title Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
Journal Reference Authors Dejgaard K, Theberge JF, Heath-Engel H, Chevet E, Tremblay ML, Thomas DY
Title Organization of the Sec61 translocon, studied by high resolution native electrophoresis.
Journal Related
