Entry
Name
Protein processing in endoplasmic reticulum - Rhinopithecus bieti (black snub-nosed monkey)
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 degradation
BRITE hierarchy
Pathway map
rbb04141 Protein processing in endoplasmic reticulum
Other DBs
Organism
Rhinopithecus bieti (black snub-nosed monkey) [GN:
rbb ]
Gene
108512121 ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586 ]
108512696 EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086 ]
108515301 DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
108516559 STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
108516893 DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518 ]
108516971 SEC31A; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005 ]
108518377 UFD1L; ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016 ]
108518882 EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860 ] [EC:2.7.11.1 ]
108519175 TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
108519685 DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452 ]
108519829 RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
108519898 ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
108520463 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
108522042 SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342 ]
108523574 DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
108523638 PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019 ]
108524470 VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
108524828 HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485 ]
108526546 UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718 ] [EC:2.4.1.-]
108526614 DNAJC10; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
108527063 BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556 ]
108527136 NFE2L2; LOW QUALITY PROTEIN: nuclear factor erythroid 2-related factor 2 [KO:K05638 ]
108527842 PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018 ]
108528732 EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
108528804 DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525 ]
108529547 SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953 ] [EC:3.6.5.-]
108529558 SKP1; LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094 ]
108530078 TRAM1; LOW QUALITY PROTEIN: translocating chain-associated membrane protein 1 [KO:K14010 ]
108531954 HSPA8; LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein [KO:K03283 ]
108532559 RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839 ]
108532710 DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525 ]
108533442 HSP90AB1; LOW QUALITY PROTEIN: heat shock protein HSP 90-beta [KO:K04079 ]
108533712 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
108533865 HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027 ]
108534121 NPLOC4; LOW QUALITY PROTEIN: nuclear protein localization protein 4 homolog [KO:K14015 ]
108534313 SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
108534646 EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085 ]
108537478 SEC61A1; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit alpha [KO:K10956 ]
108537517 RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
108538528 SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956 ]
108541796 SVIP; LOW QUALITY PROTEIN: small VCP/p97-interacting protein [KO:K14014 ]
108542298 HSP90AA1; LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha [KO:K04079 ]
108542335 STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
108542583 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
108542584 HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283 ]
108542887 MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ]
108544095 RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839 ]
108544403 RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000 ]
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 pathway
KO pathway