KEGG   PATHWAY: esp04141
Entry
esp04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Etheostoma spectabile (orangethroat darter)
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
esp04141  Protein processing in endoplasmic reticulum
esp04141

Other DBs
GO: 0030433 0034976
Organism
Etheostoma spectabile (orangethroat darter) [GN:esp]
Gene
116687894  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
116688386  protein transport protein Sec61 subunit alpha-like [KO:K10956]
116690988  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
116672051  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
116698716  sec62; translocation protein SEC62 [KO:K12275]
116706618  sec63; translocation protein SEC63 homolog [KO:K09540]
116687901  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
116688321  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
116699558  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
116692819  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
116700381  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
116701725  stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
116685712  magt1; magnesium transporter protein 1 [KO:K19478]
116698489  tumor suppressor candidate 3 [KO:K19478]
116686036  ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
116674431  transmembrane protein 258 [KO:K26502]
116694373  tmem258; transmembrane protein 258 [KO:K26502]
116704433  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
116673183  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
116705705  rrbp1; ribosome-binding protein 1 isoform X1 [KO:K14000]
116697091  sil1; LOW QUALITY PROTEIN: nucleotide exchange factor SIL1 [KO:K14001]
116700125  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
116704362  hspa5; endoplasmic reticulum chaperone BiP isoform X1 [KO:K09490] [EC:3.6.4.10]
116688988  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
116678166  dnajc1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
116697913  dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
116698080  dnaJ homolog subfamily C member 3-like [KO:K09523]
116706893  dnaJ homolog subfamily C member 3-like [KO:K09523]
116697907  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
116694661  hsp90b1; endoplasmin [KO:K09487]
116696962  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
116707280  glucosidase 2 subunit beta-like isoform X1 [KO:K08288]
116702919  prkcsh; glucosidase 2 subunit beta [KO:K08288]
116693577  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
116698613  calreticulin-like [KO:K08057]
116695809  calreticulin-like [KO:K08057]
116695827  calreticulin-like [KO:K08057]
116690453  man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
116706290  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
116689352  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
116704761  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
116686931  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
116696549  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
116704371  lman1; protein ERGIC-53 [KO:K10080]
116699434  prolactin regulatory element-binding protein-like isoform X1 [KO:K14003]
116686434  prolactin regulatory element-binding protein-like isoform X1 [KO:K14003]
116704988  GTP-binding protein SAR1b-like [KO:K07953] [EC:3.6.5.-]
116700744  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
116670798  sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
116687808  sec13; protein SEC13 homolog [KO:K14004]
116670744  protein transport protein Sec31A-like isoform X1 [KO:K14005]
116703990  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
116701208  protein transport protein Sec23A [KO:K14006]
116669438  sec24d; protein transport protein Sec24D [KO:K14007]
116700740  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
116671719  sec24c; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
116689397  protein transport protein Sec24C-like isoform X1 [KO:K14007]
116696578  protein transport protein Sec24B-like isoform X1 [KO:K14007]
116698399  uggt2; LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
116670148  LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 1-like [KO:K11718] [EC:2.4.1.-]
116687722  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
116685430  LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
116694902  LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
116703931  ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
116702656  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
116671995  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
116706355  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
116672183  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
116687128  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
116688369  os9; protein OS-9 isoform X1 [KO:K10088]
116704901  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
116687768  ssr1; translocon-associated protein subunit alpha [KO:K13249]
116702113  ssr2; translocon-associated protein subunit beta [KO:K13250]
116678739  ssr3; translocon-associated protein subunit gamma [KO:K13251]
116692345  ssr4; translocon-associated protein subunit delta [KO:K04571]
116692692  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
116672286  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
116690588  derl1; derlin-1 [KO:K11519]
116689180  derlin-2-like [KO:K13989]
116704162  derl2; derlin-2 [KO:K13989]
116683360  ubxn4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
116696964  ubxn1; UBX domain-containing protein 1 [KO:K24348]
116706609  ubxn2a; UBX domain-containing protein 2A isoform X1 [KO:K24349]
116696100  ubxn6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
116692611  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
116693896  svip; small VCP/p97-interacting protein [KO:K14014]
116700298  LOW QUALITY PROTEIN: transitional endoplasmic reticulum ATPase-like [KO:K13525]
116689243  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
116671008  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
116689744  ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
116691017  heat shock cognate 71 kDa protein-like [KO:K03283]
116692861  heat shock 70 kDa protein [KO:K03283]
116700138  heat shock cognate 70 kDa protein [KO:K03283]
116702985  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
116699459  dnaJ homolog subfamily A member 1-like [KO:K09502]
116693094  dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
116694244  dnaJ homolog subfamily A member 2-like [KO:K09503]
116700244  dnaJ homolog subfamily B member 1-like [KO:K09507]
116703688  dnaJ homolog subfamily B member 1-like [KO:K09507]
116697701  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
116705474  dnaJ homolog subfamily B member 12-like [KO:K09518]
116670776  dnajb12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
116678887  dnaJ homolog subfamily C member 5-like [KO:K09525]
116692091  dnaJ homolog subfamily C member 5-like isoform X1 [KO:K09525]
116706200  dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
116669993  dnaJ homolog subfamily C member 5-like [KO:K09525]
116670384  dnaJ homolog subfamily C member 5-like isoform X1 [KO:K09525]
116706735  hsp90aa1; heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
116670290  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
116698535  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
116705343  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
116703759  hspbp1; hsp70-binding protein 1 [KO:K09562]
116673713  cryaa; alpha-crystallin A chain [KO:K09541]
116691073  hspb6; heat shock protein beta-6 [KO:K09542]
116700045  alpha-crystallin B chain-like [KO:K09542]
116688299  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
116686578  plaa; phospholipase A-2-activating protein [KO:K14018]
116704455  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
116695119  UV excision repair protein RAD23 homolog B-like isoform X1 [KO:K10839]
116703172  UV excision repair protein RAD23 homolog A-like isoform X1 [KO:K10839]
116691176  ubiquilin-4-like [KO:K04523]
116701239  ubiquilin-4-like [KO:K04523]
116688904  ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
116693396  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
116670427  ataxin-3-like [KO:K11863] [EC:3.4.22.-]
116692982  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
116686948  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
116706045  eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
116697955  nfe2l2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
116692368  transcription factor NF-E2 45 kDa subunit-like [KO:K05638]
116700966  atf4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
116687277  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
116698657  apoptosis regulator Bcl-2-like [KO:K02161]
116696248  LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-6 alpha-like [KO:K09054]
116693033  cyclic AMP-dependent transcription factor ATF-6 beta-like [KO:K09049]
116670418  wolframin-like isoform X1 [KO:K14020]
116696817  wfs1; wolframin [KO:K14020]
116694408  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
116671951  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
116690292  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
116703426  ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
116703724  serine/threonine-protein kinase/endoribonuclease IRE1-like isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
116690145  LOW QUALITY PROTEIN: TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
116704577  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
116703984  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
116690444  mitogen-activated protein kinase kinase kinase 5-like [KO:K04426] [EC:2.7.11.25]
116706745  map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
116705548  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
116697416  mitogen-activated protein kinase 9-like [KO:K04440] [EC:2.7.11.24]
116705432  mitogen-activated protein kinase 8-like isoform X1 [KO:K04440] [EC:2.7.11.24]
116671539  mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
116703876  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
116683326  apoptosis regulator BAX-like [KO:K02159]
116686021  LOW QUALITY PROTEIN: bcl-2 homologous antagonist/killer-like [KO:K02159]
116703270  bax; apoptosis regulator BAX [KO:K02159]
116675297  apoptosis regulator BAX-like isoform X1 [KO:K02159]
116703331  apoptosis regulator BAX-like isoform X1 [KO:K02159]
116704838  calpain-1 catalytic subunit-like isoform X1 [KO:K01367] [EC:3.4.22.52]
116706951  capn1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
116669923  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
116704839  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
116698650  E3 ubiquitin-protein ligase MARCH6-like isoform X1 [KO:K10661] [EC:2.3.2.27]
116700131  LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase MARCH6-like [KO:K10661] [EC:2.3.2.27]
116672394  marchf6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
116706465  ube2j1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
116687208  ube2j2; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
116704554  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
116686663  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
116700985  ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
116689307  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
116685937  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
116701780  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
116686794  selenos; selenoprotein S [KO:K14025]
116705903  sel1l; protein sel-1 homolog 1 [KO:K14026]
116693958  homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 2 protein isoform X1 [KO:K14027]
116692584  E3 ubiquitin-protein ligase AMFR-like [KO:K10636] [EC:2.3.2.36]
116706973  E3 ubiquitin-protein ligase AMFR-like [KO:K10636] [EC:2.3.2.36]
116671212  STIP1 homology and U box-containing protein 1-like [KO:K09561] [EC:2.3.2.27]
116702994  E3 ubiquitin-protein ligase CHIP-like [KO:K09561] [EC:2.3.2.27]
116704627  ubiquitin-conjugating enzyme E2 D4-like isoform X1 [KO:K06689] [EC:2.3.2.23]
116705504  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
116679545  ubiquitin-conjugating enzyme E2-17 kDa-like [KO:K06689] [EC:2.3.2.23]
116685966  ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
116686079  ubiquitin-conjugating enzyme E2 D2-like isoform X1 [KO:K06689] [EC:2.3.2.23]
116671549  ubiquitin-conjugating enzyme E2 D4-like isoform X1 [KO:K06689] [EC:2.3.2.23]
116689290  ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
116702801  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
116679238  cul1; cullin-1 [KO:K03347]
116700182  skp1; LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094]
116688210  F-box only protein 2-like [KO:K10099]
116692164  F-box only protein 6-like isoform X1 [KO:K10100]
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
esp00510  N-Glycan biosynthesis
esp03050  Proteasome
esp03060  Protein export
esp04210  Apoptosis
KO pathway
ko04141   
LinkDB

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