Entry
Name
Protein processing in endoplasmic reticulum - Anguilla anguilla (European eel)
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
aang04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Anguilla anguilla (European eel) [GN:
aang ]
Gene
118211754 rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
118211771 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
118226039 dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
118210535 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
118207946 stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
118233778 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
118236517 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
118214107 dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530 ] [EC:1.8.4.-]
118213031 man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
118222561 lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082 ]
118224404 preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003 ]
118218085 sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953 ] [EC:3.6.5.-]
118218328 LOW QUALITY PROTEIN: protein transport protein Sec24C-like [KO:K14007 ]
118207121 si:dkey-13n15.2; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007 ]
118230853 uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
118214132 uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
118208046 edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
118208712 edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
118230460 edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
118212729 si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
118221523 ero1b; ERO1-like protein beta isoform X1 [KO:K10976 ] [EC:1.8.4.-]
118225094 translocon-associated protein subunit alpha-like isoform X1 [KO:K13249 ]
118234267 ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
118235188 translocon-associated protein subunit beta isoform X1 [KO:K13250 ]
118226172 tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
118208461 zgc:113278; translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
118234117 translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
118206559 vcp; LOW QUALITY PROTEIN: transitional endoplasmic reticulum ATPase [KO:K13525 ]
118219787 zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525 ]
118221230 nploc4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015 ]
118237525 ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016 ]
118230070 dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525 ]
118225383 rad23ab; RAD23 homolog A, nucleotide excision repair protein b [KO:K10839 ]
118213269 rad23b; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839 ]
118214352 eif2s1a; eukaryotic translation initiation factor 2 subunit 1a [KO:K03237 ]
118222305 nfe2l2a; nuclear factor erythroid 2-related factor 2a isoform X1 [KO:K05638 ]
118220191 atf4a; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
118210923 ddit3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452 ]
118230021 cyclic AMP-dependent transcription factor ATF-6 alpha-like [KO:K09054 ]
118225389 atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
118234280 atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
118228004 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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