Entry
Name
Protein processing in endoplasmic reticulum - Paramormyrops kingsleyae
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
pki04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Paramormyrops kingsleyae [GN:
pki ]
Gene
111837166 sec61g; protein transport protein Sec61 subunit gamma [KO:K07342 ]
111856684 sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540 ]
111832900 rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
111840978 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
111846226 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1 [KO:K12668 ]
111857164 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
111853729 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
111857844 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
111849584 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
111846954 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
111838607 dnaJ homolog subfamily C member 10-like isoform X1 [KO:K09530 ] [EC:1.8.4.-]
111834451 dnaJ homolog subfamily C member 10-like isoform X1 [KO:K09530 ] [EC:1.8.4.-]
111854001 endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
111857531 man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
111838655 preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003 ]
111855021 LOW QUALITY PROTEIN: protein transport protein Sec23A-like [KO:K14006 ]
111847416 uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
111859683 UDP-glucose:glycoprotein glucosyltransferase 2-like isoform X1 [KO:K11718 ] [EC:2.4.1.-]
111840537 edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084 ]
111837011 edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
111860257 edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
111853977 ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086 ]
111836496 ero1a; ERO1-like protein alpha isoform X1 [KO:K10950 ] [EC:1.8.4.-]
111856368 ero1b; ERO1-like protein beta isoform X1 [KO:K10976 ] [EC:1.8.4.-]
111858968 ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
111837917 ssr2; translocon-associated protein subunit beta isoform X1 [KO:K13250 ]
111834935 tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
111857357 translocating chain-associated membrane protein 1-like [KO:K14010 ]
111840957 transitional endoplasmic reticulum ATPase-like isoform X1 [KO:K13525 ]
111838289 nuclear protein localization protein 4 homolog isoform X1 [KO:K14015 ]
111856849 ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016 ]
111854020 dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525 ]
111838466 UV excision repair protein RAD23 homolog B-like isoform X1 [KO:K10839 ]
111834239 UV excision repair protein RAD23 homolog B-like isoform X1 [KO:K10839 ]
111858978 eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
111838582 nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638 ]
111841038 atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
111840080 cyclic AMP-dependent transcription factor ATF-6 alpha-like [KO:K09054 ]
111842807 atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
111845540 ern2; serine/threonine-protein kinase/endoribonuclease IRE2 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
111848220 ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
111851896 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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