Entry
Name
Protein processing in endoplasmic reticulum - Sinocyclocheilus grahami (golden-line barbel)
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
sgh04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Sinocyclocheilus grahami (golden-line barbel) [GN:
sgh ]
Gene
107585114 protein transport protein Sec61 subunit alpha-like 1 isoform X1 [KO:K10956 ]
107593687 protein transport protein Sec61 subunit beta-like isoform X1 [KO:K09481 ]
107573146 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1-like [KO:K12666 ]
107566443 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1-like [KO:K12666 ]
107560427 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2-like [KO:K12667 ]
107557908 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
107600203 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1-like [KO:K12668 ]
107558269 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1 [KO:K12668 ]
107593933 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
107591123 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit-like [KO:K12670 ]
107551195 stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
107583387 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
107565263 LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A-like [KO:K07151 ] [EC:2.4.99.18 ]
107601384 dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
107598312 lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082 ]
107555197 UDP-glucose:glycoprotein glucosyltransferase 2-like [KO:K11718 ] [EC:2.4.1.-]
107602600 UDP-glucose:glycoprotein glucosyltransferase 1-like [KO:K11718 ] [EC:2.4.1.-]
107600415 UDP-glucose:glycoprotein glucosyltransferase 2-like [KO:K11718 ] [EC:2.4.1.-]
107588898 UDP-glucose:glycoprotein glucosyltransferase 1-like [KO:K11718 ] [EC:2.4.1.-]
107552651 ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
107584463 ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084 ]
107601347 edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085 ]
107585860 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
107595666 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
107584610 ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086 ]
107548759 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
107564746 ero1b; LOW QUALITY PROTEIN: ERO1-like protein beta [KO:K10976 ] [EC:1.8.4.-]
107564801 translocating chain-associated membrane protein 1-like [KO:K14010 ]
107596048 translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
107589926 tram1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010 ]
107555798 translocating chain-associated membrane protein 1-like [KO:K14010 ]
107579699 bag2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556 ]
107597871 LOW QUALITY PROTEIN: phospholipase A-2-activating protein-like [KO:K14018 ]
107567907 UV excision repair protein RAD23 homolog B-like isoform X1 [KO:K10839 ]
107563904 eukaryotic translation initiation factor 2 subunit 1-like [KO:K03237 ]
107559120 eukaryotic translation initiation factor 2 subunit 1-like [KO:K03237 ]
107568209 eukaryotic translation initiation factor 2 subunit 1-like [KO:K03237 ]
107568392 atf4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374 ]
107585034 cyclic AMP-dependent transcription factor ATF-4-like isoform X1 [KO:K04374 ]
107566410 ddit3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452 ]
107601246 cyclic AMP-dependent transcription factor ATF-6 alpha-like isoform X1 [KO:K09054 ]
107555632 cyclic AMP-dependent transcription factor ATF-6 alpha-like [KO:K09054 ]
107567815 cyclic AMP-dependent transcription factor ATF-6 beta-like isoform X1 [KO:K09049 ]
107566938 cyclic AMP-dependent transcription factor ATF-6 beta-like [KO:K09049 ]
107563253 serine/threonine-protein kinase/endoribonuclease ire-1-like isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
107583788 homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027 ]
107549637 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
All DBs