Entry
Name
Protein processing in endoplasmic reticulum - Rana temporaria (common frog)
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
rtem04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Rana temporaria (common frog) [GN:
rtem ]
Gene
120945103 SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956 ]
120940363 SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342 ]
120945096 RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
120918768 RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
120930312 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1 [KO:K12668 ]
120940642 DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
120924138 DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
120940314 STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
120915701 STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
120943944 DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
120914028 MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
120932419 SEC24A; LOW QUALITY PROTEIN: protein transport protein Sec24A [KO:K14007 ]
120926313 UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
120936396 UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
120944892 EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
120919520 ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
120945551 EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
120921059 ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950 ] [EC:1.8.4.-]
120940689 SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
120942680 LOW QUALITY PROTEIN: translocon-associated protein subunit beta-like [KO:K13250 ]
120941116 TRAM1; translocating chain-associated membrane protein 1 [KO:K14010 ]
120919024 NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015 ]
120936123 UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016 ]
120941070 DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525 ]
120945185 RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839 ]
120921192 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
120945302 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
120945523 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
120913240 ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
120917700 HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [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
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