KEGG   PATHWAY: pfor04141
Entry
pfor04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Poecilia formosa (Amazon molly)
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
pfor04141  Protein processing in endoplasmic reticulum
pfor04141

Other DBs
GO: 0030433 0034976
Organism
Poecilia formosa (Amazon molly) [GN:pfor]
Gene
103145786  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
103143230  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
103140873  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
107835785  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
103146083  sec62; translocation protein SEC62 [KO:K12275]
103138681  sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
103145772  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
103144071  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
103144103  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2-like isoform X2 [KO:K12667]
103138430  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
103150462  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
103144446  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
103142125  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
103136961  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
103148419  tumor suppressor candidate 3-like [KO:K19478]
103148420  tumor suppressor candidate 3 [KO:K19478]
103130314  magnesium transporter protein 1 isoform X1 [KO:K19478]
103143401  ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
103141294  tmem258; transmembrane protein 258 [KO:K26502]
103148467  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
103156935  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
103134148  rrbp1; ribosome-binding protein 1 isoform X3 [KO:K14000]
103131634  nucleotide exchange factor SIL1-like [KO:K14001]
103156532  nucleotide exchange factor SIL1-like [KO:K14001]
103132034  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
103150004  hspa5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
103156707  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
103131111  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
103137566  dnaJ homolog subfamily C member 3-like [KO:K09523]
103134259  dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
103151392  dnaJ homolog subfamily C member 10-like [KO:K09530] [EC:1.8.4.-]
103151393  dnajc10; dnaJ homolog subfamily C member 10 isoform X2 [KO:K09530] [EC:1.8.4.-]
103130433  hsp90b1; endoplasmin [KO:K09487]
103136895  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
103145194  glucosidase 2 subunit beta-like isoform X1 [KO:K08288]
103143747  prkcsh; glucosidase 2 subunit beta [KO:K08288]
103130783  canx; calnexin isoform X1 [KO:K08054]
103152828  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
103147914  protein disulfide-isomerase A3-like [KO:K08056] [EC:5.3.4.1]
103138474  calreticulin [KO:K08057]
103146065  calreticulin-like [KO:K08057]
103152143  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
103139292  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
103152699  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
103141937  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like [KO:K23741] [EC:3.2.1.209]
103155541  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
103140086  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
103142726  lman1; protein ERGIC-53 isoform X1 [KO:K10080]
103139955  preb; prolactin regulatory element-binding protein [KO:K14003]
103133492  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
103142298  GTP-binding protein SAR1b-like [KO:K07953] [EC:3.6.5.-]
103155754  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
103150140  sec13; protein SEC13 homolog [KO:K14004]
103151007  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
103142290  protein transport protein Sec31A-like isoform X1 [KO:K14005]
103131642  protein transport protein Sec23B [KO:K14006]
103131658  protein transport protein Sec23A-like [KO:K14006]
103153844  protein transport protein Sec23A isoform X2 [KO:K14006]
103145142  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
103152183  protein transport protein Sec24B isoform X1 [KO:K14007]
103143418  sec24d; protein transport protein Sec24D [KO:K14007]
103134904  protein transport protein Sec24C-like isoform X1 [KO:K14007]
103155753  sec24a; protein transport protein Sec24A isoform X4 [KO:K14007]
103134260  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
103155207  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
103133112  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
103156225  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
103147723  ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X3 [KO:K10086]
103142533  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
103133285  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
103135239  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
103134176  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
103135300  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
103142645  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
103146658  ero1b; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
103147664  protein OS-9-like isoform X1 [KO:K10088]
103147676  protein OS-9-like [KO:K10088]
103134099  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
103144307  translocon-associated protein subunit alpha-like [KO:K13249]
103156751  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
103142794  ssr2; translocon-associated protein subunit beta [KO:K13250]
103153977  ssr3; translocon-associated protein subunit gamma [KO:K13251]
103141161  ssr4; translocon-associated protein subunit delta [KO:K04571]
103130521  bcap31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
103139772  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
103147800  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
103142137  derl1; derlin-1 [KO:K11519]
103131304  derl2; derlin-2 [KO:K13989]
103134911  derlin-2-like isoform X1 [KO:K13989]
103154102  ubxn4; UBX domain-containing protein 4 [KO:K24348]
103136897  ubxn1; UBX domain-containing protein 1 [KO:K24348]
103148032  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
103155175  ubxn6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
103156118  nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
103156507  svip; small VCP/p97-interacting protein [KO:K14014]
103154164  transitional endoplasmic reticulum ATPase-like isoform X2 [KO:K13525]
103142927  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
103152687  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
103145837  ufd1l; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
103130283  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
103151051  heat shock 70 kDa protein-like [KO:K03283]
103132027  heat shock 70 kDa protein 1 [KO:K03283]
103139657  heat shock cognate 71 kDa protein-like [KO:K03283]
103139658  heat shock cognate 71 kDa protein-like [KO:K03283]
103137846  dnaJ homolog subfamily A member 1-like [KO:K09502]
103137851  dnaJ homolog subfamily A member 1-like [KO:K09502]
103131486  dnaJ homolog subfamily A member 2 [KO:K09503]
103133785  dnaJ homolog subfamily A member 2-like [KO:K09503]
103151990  dnaJ homolog subfamily B member 1-like [KO:K09507]
103154160  dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
103140760  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
103133487  dnaJ homolog subfamily B member 12-like [KO:K09518]
103142293  dnaJ homolog subfamily B member 12-like [KO:K09518]
103144709  dnaJ homolog subfamily C member 5-like isoform X1 [KO:K09525]
103139084  dnaJ homolog subfamily C member 5 [KO:K09525]
103148186  dnaJ homolog subfamily C member 5-like [KO:K09525]
103148188  dnaJ homolog subfamily C member 5-like [KO:K09525]
103129223  dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
103129934  dnajc5b; dnaJ homolog subfamily C member 5B [KO:K09525]
103133694  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
103155532  heat shock protein HSP 90-alpha [KO:K04079]
103155534  heat shock protein HSP 90-alpha 1 [KO:K04079]
103154766  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
103135690  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
103134079  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
103145415  hspbp1; hsp70-binding protein 1 [KO:K09562]
103149040  alpha-crystallin A chain [KO:K09541]
103141149  alpha-crystallin B chain [KO:K09542]
103143109  hspb6; heat shock protein beta-6 [KO:K09542]
103134639  ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
103134641  ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
103143389  plaa; phospholipase A-2-activating protein isoform X1 [KO:K14018]
103152589  rad23a; LOW QUALITY PROTEIN: UV excision repair protein RAD23 homolog A [KO:K10839]
103142755  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
103135083  ubqln4; ubiquilin-4 isoform X1 [KO:K04523]
103135467  ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
103142397  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X3 [KO:K01456] [EC:3.5.1.52]
103138792  ataxin-3-like isoform X1 [KO:K11863] [EC:3.4.22.-]
103138910  ube4b; ubiquitin conjugation factor E4 B isoform X2 [KO:K10597] [EC:2.3.2.27]
103133060  eukaryotic translation initiation factor 2-alpha kinase 3-like [KO:K08860] [EC:2.7.11.1]
103149415  eukaryotic translation initiation factor 2-alpha kinase 3-like [KO:K08860] [EC:2.7.11.1]
103133672  eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
103134325  nfe2l2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
103147384  cyclic AMP-dependent transcription factor ATF-4-like isoform X1 [KO:K04374]
103154846  cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
103135803  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
103143027  bcl2; apoptosis regulator Bcl-2 [KO:K02161]
103142583  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
103149745  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
103137797  wolframin-like isoform X1 [KO:K14020]
103147457  wfs1; wolframin [KO:K14020]
103144371  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
103140524  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
103134937  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
103130409  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103141628  ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103154502  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
103141905  TNF receptor-associated factor 2-like isoform X1 [KO:K03173] [EC:2.3.2.27]
103149802  traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
103154474  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
103141536  mitogen-activated protein kinase kinase kinase 5-like [KO:K04426] [EC:2.7.11.25]
103153184  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
103134016  mitogen-activated protein kinase 8-like isoform X1 [KO:K04440] [EC:2.7.11.24]
103135541  mitogen-activated protein kinase 9 isoform X2 [KO:K04440] [EC:2.7.11.24]
103142718  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
103143577  mapk8; mitogen-activated protein kinase 8 isoform X2 [KO:K04440] [EC:2.7.11.24]
103150954  apoptosis regulator BAX-like [KO:K02159]
103142241  apoptosis regulator BAX-like [KO:K02159]
103142242  apoptosis regulator BAX-like [KO:K02159]
103130122  calpain-1 catalytic subunit-like isoform X1 [KO:K01367] [EC:3.4.22.52]
103145921  calpain-1 catalytic subunit-like isoform X2 [KO:K01367] [EC:3.4.22.52]
103134144  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
103154184  calpain-8-like [KO:K01367] [EC:3.4.22.52]
103154186  calpain-A-like [KO:K01367] [EC:3.4.22.52]
103130124  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
103134145  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
103134146  calpain-2 catalytic subunit-like isoform X1 [KO:K03853] [EC:3.4.22.53]
103135218  march6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
103150850  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
103139501  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
103152401  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
103142078  ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
103153902  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
103149302  syvn1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
103154568  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
103148904  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
103149189  vimp; selenoprotein S [KO:K14025]
103144173  protein sel-1 homolog 1-like [KO:K14026]
103144174  protein sel-1 homolog 1-like [KO:K14026]
103142462  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
103142810  amfr; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
103156087  STIP1 homology and U box-containing protein 1-like [KO:K09561] [EC:2.3.2.27]
103156089  STIP1 homology and U box-containing protein 1-like [KO:K09561] [EC:2.3.2.27]
103131211  ube2d3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
103146202  ubiquitin-conjugating enzyme E2 D4 isoform X2 [KO:K06689] [EC:2.3.2.23]
103133599  ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
103149268  ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
103150060  ubiquitin-conjugating enzyme E2 D2-like isoform X2 [KO:K06689] [EC:2.3.2.23]
103130874  park2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
103153424  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
103131052  cul1; cullin-1 [KO:K03347]
103155759  skp1; S-phase kinase-associated protein 1 [KO:K03094]
103144087  F-box only protein 2-like [KO:K10099]
103144057  uncharacterized protein LOC103144057 [KO:K10100]
103144056  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
pfor00510  N-Glycan biosynthesis
pfor03050  Proteasome
pfor03060  Protein export
pfor04210  Apoptosis
KO pathway
ko04141   
LinkDB

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