Entry
Name
Protein processing in endoplasmic reticulum - Rousettus aegyptiacus (Egyptian rousette)
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
ray04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Rousettus aegyptiacus (Egyptian rousette) [GN:
ray ]
Gene
107503655 SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956 ]
107504854 SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 isoform X1 [KO:K10956 ]
107521435 LOW QUALITY PROTEIN: protein transport protein Sec61 subunit gamma-like [KO:K07342 ]
107501318 SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342 ]
107497052 RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666 ]
107512601 RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
107503908 DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
107513042 DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
107498937 STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
107508788 STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
107503848 MAGT1; LOW QUALITY PROTEIN: magnesium transporter protein 1 [KO:K19478 ]
107505074 CKAP4; LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999 ]
107499908 DNAJC1; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1 [KO:K09521 ]
107513285 DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
107499772 SEC31B; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005 ]
107516883 UGGT1; LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718 ] [EC:2.4.1.-]
107517651 UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
107504150 EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084 ]
107500804 EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
107511305 EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
107518910 SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
107504702 SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571 ]
107514829 TRAM1; translocating chain-associated membrane protein 1 [KO:K14010 ]
107510890 TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
107502827 VCP; LOW QUALITY PROTEIN: transitional endoplasmic reticulum ATPase [KO:K13525 ]
107520209 NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015 ]
107521846 UFD1L; ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016 ]
107498470 LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283 ]
107502754 DNAJA1; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1 [KO:K09502 ]
107519523 DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518 ]
107502134 DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525 ]
107503258 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
107514669 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638 ]
107500816 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
107498869 PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019 ]
107502472 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
107498439 ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
107502023 ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
107501913 HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027 ]
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
LinkDB
All DBs