Entry
Name
Protein processing in endoplasmic reticulum - Anser cygnoides (swan goose)
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
acyg04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Anser cygnoides (swan goose) [GN:
acyg ]
Gene
106046212 SEC61A2; protein transport protein Sec61 subunit alpha isoform X2 [KO:K10956 ]
106038064 SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956 ]
106046414 SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342 ]
106037921 RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
106040863 RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
136786829 DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
106048632 DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
106040606 STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
106043315 STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
106030053 DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
106037118 MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
106049898 PREB; guanine nucleotide-exchange factor SEC12 isoform X1 [KO:K14003 ]
106049340 UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
106040318 UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
106036703 EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
106047756 EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
106034049 EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
106044015 ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950 ] [EC:1.8.4.-]
106048088 ERO1B; ERO1-like protein beta isoform X1 [KO:K10976 ] [EC:1.8.4.-]
106034853 SSR1; translocon-associated protein subunit alpha isoform X2 [KO:K13249 ]
136787432 BCAP31; LOW QUALITY PROTEIN: B-cell receptor-associated protein 31 [KO:K14009 ]
106030605 TRAM1; translocating chain-associated membrane protein 1 [KO:K14010 ]
106041315 translocating chain-associated membrane protein 1-like 1 isoform X1 [KO:K14010 ]
106049410 NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015 ]
106031252 UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016 ]
136788154 LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502 ]
125181512 DNAJB1; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1 [KO:K09507 ]
106045014 BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555 ]
106040437 RAD23B; UV excision repair protein RAD23 homolog B isoform X3 [KO:K10839 ]
106031680 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
106030027 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X2 [KO:K05638 ]
106035925 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
106041594 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
125181848 MAP2K7; LOW QUALITY PROTEIN: dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431 ] [EC:2.7.12.2 ]
106033143 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
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