KEGG   PATHWAY: aang04141
Entry
aang04141                   Pathway                                
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
Pathway map
aang04141  Protein processing in endoplasmic reticulum
aang04141

Other DBs
GO: 0030433 0034976
Organism
Anguilla anguilla (European eel) [GN:aang]
Gene
118206153  [KO:K10839]
118206207  [KO:K03173] [EC:2.3.2.27]
118206271  derl3; derlin-3 isoform X1 [KO:K13989]
118206559  vcp; LOW QUALITY PROTEIN: transitional endoplasmic reticulum ATPase [KO:K13525]
118206675  [KO:K09490] [EC:5.6.1.10]
118206731  ube2g1b; ubiquitin-conjugating enzyme E2G 1b [KO:K10575] [EC:2.3.2.23]
118206743  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
118206891  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
118207121  si:dkey-13n15.2; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
118207304  hspa1b; heat shock 70 kDa protein 1B [KO:K03283]
118207623  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
118207640  [KO:K14021]
118207946  stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
118207966  ssr4; translocon-associated protein subunit delta [KO:K04571]
118208046  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
118208232  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
118208426  [KO:K09525]
118208461  zgc:113278; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
118208703  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
118208712  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
118208823  hsph1; heat shock protein 105 kDa [KO:K09485]
118208951  [KO:K03283]
118209297  cryaba; crystallin, alpha B, a [KO:K09542]
118209898  ssr3; translocon-associated protein subunit gamma [KO:K13251]
118210248  [KO:K10575] [EC:2.3.2.23]
118210421  [KO:K07151] [EC:2.4.99.18]
118210505  [KO:K14012]
118210535  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
118210923  ddit3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
118210936  os9; protein OS-9 isoform X1 [KO:K10088]
118211040  fbxo2; F-box only protein 2 isoform X1 [KO:K10099]
118211159  [KO:K03283]
118211160  [KO:K03283]
118211167  [KO:K09525]
118211230  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
118211285  sec13; protein SEC13 homolog [KO:K14004]
118211551  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
118211581  [KO:K10956]
118211754  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
118211771  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
118211935  [KO:K13249]
118212094  [KO:K09490] [EC:5.6.1.10]
118212625  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
118212729  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
118212863  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
118212877  [KO:K03853] [EC:3.4.22.53]
118212878  [KO:K03853] [EC:3.4.22.53]
118212933  traf2b; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
118213001  [KO:K13525]
118213031  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
118213053  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
118213064  ubxn8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
118213085  lman1; protein ERGIC-53 [KO:K10080]
118213211  [KO:K03173] [EC:2.3.2.27]
118213269  rad23b; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
118213512  [KO:K04079]
118213849  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
118214083  [KO:K01230] [EC:3.2.1.113]
118214107  dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
118214132  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
118214152  [KO:K09541]
118214352  eif2s1a; eukaryotic translation initiation factor 2 subunit 1a [KO:K03237]
118214577  [KO:K24348]
118214689  zgc:153993; apoptosis regulator BAX [KO:K02159]
118214737  selenos; selenoprotein S [KO:K14025]
118215183  [KO:K09503]
118215281  [KO:K10636] [EC:2.3.2.36]
118215940  prkcsh; glucosidase 2 subunit beta [KO:K08288]
118216522  hspbp1; hsp70-binding protein 1 isoform X1 [KO:K09562]
118216689  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
118216810  [KO:K09561] [EC:2.3.2.27]
118216823  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118217257  atf4b; activating transcription factor 4b isoform X1 [KO:K04374]
118217377  baxa; BCL2 associated X, apoptosis regulator a [KO:K02159]
118217557  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
118217731  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
118217792  capn1a; calpain 1, (mu/I) large subunit a [KO:K01367] [EC:3.4.22.52]
118217793  capn2a; calpain 2, (m/II) large subunit a [KO:K03853] [EC:3.4.22.53]
118217861  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
118218059  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
118218075  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
118218085  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
118218150  prkn; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
118218222  [KO:K06689] [EC:2.3.2.23]
118218248  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
118218328  [KO:K14007]
118218445  [KO:K03853] [EC:3.4.22.53]
118218446  capn8; calpain-8 [KO:K03853] [EC:3.4.22.53]
118218515  [KO:K03868] [EC:2.3.2.32]
118218768  [KO:K09487]
118218853  [KO:K28771]
118219098  [KO:K09518]
118219227  [KO:K13999]
118219480  rrbp1b; ribosome-binding protein 1b isoform X1 [KO:K14000]
118219612  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
118219756  [KO:K02159]
118219785  [KO:K09507]
118219787  zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525]
118220191  atf4a; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
118220277  [KO:K04079]
118220367  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118220605  [KO:K09561] [EC:2.3.2.27]
118220681  [KO:K01367] [EC:3.4.22.52]
118220682  [KO:K03853] [EC:3.4.22.53]
118220706  pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
118220848  mapk8b; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
118220904  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
118220959  [KO:K03853] [EC:3.4.22.53]
118221068  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
118221077  [KO:K01367] [EC:3.4.22.52]
118221083  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
118221131  [KO:K02159]
118221230  nploc4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
118221370  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
118221523  ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
118221874  sec23b; protein transport protein Sec23B [KO:K14006]
118222305  nfe2l2a; nuclear factor erythroid 2-related factor 2a isoform X1 [KO:K05638]
118222345  sec23a; protein transport protein Sec23A [KO:K14006]
118222561  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
118222942  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
118222972  [KO:K09508]
118223007  canx; calnexin isoform X1 [KO:K08054]
118223049  [KO:K03094]
118223126  [KO:K24348]
118223310  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
118223379  cryaa; alpha-crystallin A chain [KO:K09541]
118223384  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
118223469  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
118223480  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
118223559  [KO:K07953] [EC:3.6.5.-]
118223663  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
118223879  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
118224074  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
118224128  [KO:K19478]
118224167  sil1; nucleotide exchange factor SIL1 [KO:K14001]
118224209  dnajc3a; dnaJ homolog subfamily C member 3a [KO:K09523]
118224362  [KO:K01230] [EC:3.2.1.113]
118224404  preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
118224743  [KO:K09582] [EC:5.3.4.1]
118224866  [KO:K14011]
118224917  [KO:K07342]
118225094  [KO:K13249]
118225180  [KO:K03347]
118225383  rad23ab; RAD23 homolog A, nucleotide excision repair protein b [KO:K10839]
118225385  calr3a; calreticulin 3a isoform X1 [KO:K08057]
118225389  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
118225426  [KO:K02161]
118225715  march6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
118225997  [KO:K14026]
118226039  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
118226172  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
118226407  dnajc5b; dnaJ homolog subfamily C member 5 [KO:K09525]
118226448  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
118226613  mbtps2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
118226730  tusc3; tumor suppressor candidate 3 [KO:K19478]
118226767  tmem258; transmembrane protein 258 [KO:K26502]
118226796  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
118227147  [KO:K14020]
118227281  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
118227680  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
118227698  [KO:K10636] [EC:2.3.2.36]
118227764  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
118227963  [KO:K06689] [EC:2.3.2.23]
118227979  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
118228004  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
118228297  [KO:K14007]
118228390  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
118228426  svip; small VCP/p97-interacting protein isoform X1 [KO:K14014]
118228452  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
118228809  sec63; translocation protein SEC63 homolog [KO:K09540]
118228893  [KO:K08057]
118229338  [KO:K04079]
118229339  [KO:K04079]
118229405  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
118229588  [KO:K14011]
118229979  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
118230021  [KO:K09054]
118230026  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
118230070  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
118230386  sec62; translocation protein SEC62 [KO:K12275]
118230453  [KO:K14026]
118230460  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
118230513  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
118230661  [KO:K08057]
118230705  ost4; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 4 [KO:K00730]
118230802  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
118230853  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
118231137  [KO:K09502]
118231440  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
118231559  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
118231769  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
118231966  [KO:K14020]
118231983  [KO:K14007]
118232035  [KO:K06689] [EC:2.3.2.23]
118232300  [KO:K08288]
118232374  [KO:K05545 K28771] [EC:1.3.1.90]
118232378  hsp90b1; endoplasmin [KO:K09487]
118232511  plaa; phospholipase A-2-activating protein [KO:K14018]
118232764  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
118232885  [KO:K07342]
118233019  ssr2; translocon-associated protein subunit beta [KO:K13250]
118233044  [KO:K03347]
118233045  [KO:K09582] [EC:5.3.4.1]
118233119  ubqln4; ubiquilin-4 [KO:K04523]
118233200  derl1; derlin-1 [KO:K11519]
118233778  [KO:K07151] [EC:2.4.99.18]
118233789  [KO:K02161]
118233903  dnajc1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
118234020  [KO:K09525]
118234117  [KO:K14010]
118234267  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
118234280  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
118234407  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
118234425  [KO:K10661] [EC:2.3.2.27]
118234671  pdia7; protein disulfide isomerase family A, member 7 [KO:K08056] [EC:5.3.4.1]
118234729  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
118234955  skp1; S-phase kinase-associated protein 1 [KO:K03094]
118234986  si:dkeyp-50d11.2; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
118235121  cryabb; crystallin, alpha B, b [KO:K09542]
118235188  [KO:K13250]
118235492  [KO:K01230] [EC:3.2.1.113]
118235538  [KO:K08054]
118235647  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
118235648  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
118235660  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
118235937  [KO:K03283]
118235938  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
118236028  [KO:K06689] [EC:2.3.2.23]
118236102  derl2; derlin-2 [KO:K13989]
118236139  [KO:K04440] [EC:2.7.11.24]
118236517  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
118236619  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
118237114  [KO:K04523]
118237521  man1b1b; mannosidase, alpha, class 1B, member 1b isoform X1 [KO:K23741]
118237525  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
118237571  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
Compound
C00076  Calcium cation
G00009  
G00010  
G00011  
G00012  
G10694  
Reference
PMID:19491040
  Authors
Naidoo N
  Title
ER and aging-Protein folding and the ER stress response.
  Journal
Ageing Res Rev 8:150-9 (2009)
DOI:10.1016/j.arr.2009.03.001
Reference
PMID:18023214
  Authors
Malhotra JD, Kaufman RJ
  Title
The endoplasmic reticulum and the unfolded protein response.
  Journal
Semin Cell Dev Biol 18:716-31 (2007)
DOI:10.1016/j.semcdb.2007.09.003
Reference
PMID:20347046
  Authors
Maattanen P, Gehring K, Bergeron JJ, Thomas DY
  Title
Protein quality control in the ER: the recognition of misfolded proteins.
  Journal
Semin Cell Dev Biol 21:500-11 (2010)
DOI:10.1016/j.semcdb.2010.03.006
Reference
PMID:20219571
  Authors
Stolz A, Wolf DH
  Title
Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
  Journal
Biochim Biophys Acta 1803:694-705 (2010)
DOI:10.1016/j.bbamcr.2010.02.005
Reference
PMID:20112977
  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
J Proteome Res 9:1763-71 (2010)
DOI:10.1021/pr900900x
Related
pathway
aang00510  N-Glycan biosynthesis
aang03050  Proteasome
aang03060  Protein export
aang04210  Apoptosis
KO pathway
ko04141   

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