KEGG   PATHWAY: omc04141
Entry
omc04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Onychostoma macrolepis
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
omc04141  Protein processing in endoplasmic reticulum
omc04141

Other DBs
GO: 0030433 0034976
Organism
Onychostoma macrolepis [GN:omc]
Gene
131520396  hsph1; heat shock protein 105 kDa isoform X1 [KO:K09485]
131520690  dnajc1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
131520744  marchf6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
131520893  cryaba; crystallin, alpha B, a [KO:K09542]
131521209  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
131521220  stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
131521476  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
131521600  [KO:K01230] [EC:3.2.1.113]
131521656  ubqln4; LOW QUALITY PROTEIN: ubiquilin-4 [KO:K04523]
131521725  pdia7; protein disulfide isomerase family A, member 7 [KO:K08056] [EC:5.3.4.1]
131521791  preb; prolactin regulatory element-binding protein [KO:K14003]
131522140  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
131522440  derl1; derlin-1 isoform X1 [KO:K11519]
131522492  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
131522710  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
131523114  eif2s1a; eukaryotic translation initiation factor 2 subunit 1a [KO:K03237]
131523156  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
131523197  sec62; translocation protein SEC62 [KO:K12275]
131523225  [KO:K01367] [EC:3.4.22.52]
131523233  ube2d1a; ubiquitin-conjugating enzyme E2 D1a [KO:K06689] [EC:2.3.2.23]
131523295  [KO:K01367] [EC:3.4.22.52]
131523340  sec23a; LOW QUALITY PROTEIN: protein transport protein Sec23A [KO:K14006]
131523448  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b [KO:K09525]
131523754  hspa4l; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
131524304  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
131524385  ssr3; translocon-associated protein subunit gamma [KO:K13251]
131524638  cul1a; cullin-1a [KO:K03347]
131524777  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
131524842  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
131525146  [KO:K13250]
131525276  [KO:K13250]
131525402  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
131525451  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
131525538  rnf5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
131525669  dnajb1b; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1b [KO:K09507]
131526124  [KO:K07151] [EC:2.4.99.18]
131526536  ubxn2a; UBX domain-containing protein 2A isoform X1 [KO:K24349]
131526543  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
131526591  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
131526592  [KO:K04079]
131526709  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
131526792  pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
131526886  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
131527006  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
131527039  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
131527229  sel1l; protein sel-1 homolog 1 isoform X1 [KO:K14026]
131527234  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
131527296  sec63; translocation protein SEC63 homolog [KO:K09540]
131527657  calr3b; calreticulin 3b [KO:K08057]
131527682  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
131527708  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
131528458  si:dkeyp-50d11.2; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
131528511  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
131528517  skp1; S-phase kinase-associated protein 1 [KO:K03094]
131528658  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
131528918  ube2g1b; ubiquitin-conjugating enzyme E2G 1b [KO:K10575] [EC:2.3.2.23]
131528988  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
131529058  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
131529093  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
131529141  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
131529246  sec24a; protein transport protein Sec24A [KO:K14007]
131529248  [KO:K07953] [EC:3.6.5.-]
131529463  lman1; protein ERGIC-53 [KO:K10080]
131529606  rrbp1b; ribosome-binding protein 1b isoform X1 [KO:K14000]
131529777  bag1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
131530468  rad23ab; RAD23 homolog A, nucleotide excision repair protein b [KO:K10839]
131530469  calr3a; calreticulin 3a [KO:K08057]
131530494  ubxn6; UBX domain-containing protein 6 [KO:K14011]
131530728  sec13; protein SEC13 homolog [KO:K14004]
131530830  capn8; calpain-8 [KO:K03853] [EC:3.4.22.53]
131531019  capn1b; calpain-1 catalytic subunit b [KO:K01367] [EC:3.4.22.52]
131531102  capn2l; calpain 2, (m/II) large subunit, like [KO:K03853] [EC:3.4.22.53]
131531156  [KO:K03853] [EC:3.4.22.53]
131531529  [KO:K10100]
131531554  [KO:K10100]
131531904  dnajc5ab; dnaJ homolog subfamily C member 5 [KO:K09525]
131532020  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
131532086  ssr4; translocon-associated protein subunit delta [KO:K04571]
131532089  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
131532341  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
131532622  [KO:K12668]
131532628  ube4b; LOW QUALITY PROTEIN: ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
131532658  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
131532695  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
131532873  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
131532924  dnajc5b; dnaJ homolog subfamily C member 5 [KO:K09525]
131532946  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
131532967  cul1b; cullin-1b [KO:K03347]
131532969  pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
131533054  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
131533091  bcl2a; apoptosis regulator Bcl-2a [KO:K02161]
131533094  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
131533703  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
131533760  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
131534299  amfra; autocrine motility factor receptor a isoform X1 [KO:K10636] [EC:2.3.2.36]
131534386  svip; small VCP/p97-interacting protein [KO:K14014]
131534474  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
131534693  tmem258; transmembrane protein 258 [KO:K26502]
131536189  [KO:K02159]
131536932  ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
131537345  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
131537404  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
131537441  prkcsh; glucosidase 2 subunit beta [KO:K08288]
131537527  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
131537999  [KO:K03283]
131538058  atf4b; activating transcription factor 4b [KO:K04374]
131538287  bcap29; LOW QUALITY PROTEIN: B-cell receptor-associated protein 29 [KO:K05545 K28771] [EC:1.3.1.90]
131538927  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
131539526  zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525]
131540102  traf2b; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
131540159  rnf185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
131540213  ganaba; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
131540408  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
131540418  hspa5; LOW QUALITY PROTEIN: endoplasmic reticulum chaperone BiP [KO:K09490] [EC:5.6.1.10]
131540435  derl2; derlin-2 [KO:K13989]
131540439  traf2a; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
131540666  man1b1b; mannosidase, alpha, class 1B, member 1b [KO:K23741]
131540710  [KO:K03173] [EC:2.3.2.27]
131541096  cryabb; crystallin, alpha B, b [KO:K09542]
131541438  [KO:K09490] [EC:5.6.1.10]
131541439  [KO:K09490] [EC:5.6.1.10]
131541575  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
131541677  si:dkey-13n15.2; protein transport protein Sec24C isoform X1 [KO:K14007]
131541690  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
131541727  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
131542216  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
131542235  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
131542362  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
131542471  os9; protein OS-9 isoform X1 [KO:K10088]
131542752  wfs1a; LOW QUALITY PROTEIN: wolframin [KO:K14020]
131542783  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
131542904  sec61a1a; protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
131542929  sec61a1b; protein transport protein Sec61 subunit alpha-like 2 [KO:K10956]
131542936  atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
131543008  sec23b; protein transport protein Sec23B [KO:K14006]
131543607  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
131543737  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
131544060  plaa; phospholipase A-2-activating protein [KO:K14018]
131544571  [KO:K10100]
131544583  sec24d; protein transport protein Sec24D isoform X1 [KO:K14007]
131544617  cryaa; alpha-crystallin A chain [KO:K09541]
131544625  zgc:153993; apoptosis regulator BAX [KO:K02159]
131544799  capn1; LOW QUALITY PROTEIN: calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
131545015  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
131545341  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
131545360  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
131545427  calr; calreticulin [KO:K08057]
131545504  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
131545864  hspa1b; heat shock 70 kDa protein-like [KO:K03283]
131545985  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
131546014  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
131546041  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
131546289  tusc3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
131546456  dnajc10; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
131546689  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
131546742  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
131546966  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
131546999  ubxn4; UBX domain-containing protein 4 [KO:K24348]
131547064  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
131547065  dnajc3a; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 3a [KO:K09523]
131547328  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
131547728  ubxn8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
131548235  mogs; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
131548509  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
131548586  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
131548611  hyou1; LOW QUALITY PROTEIN: hypoxia up-regulated protein 1 [KO:K09486]
131548732  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
131549303  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
131549410  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
131549527  [KO:K04554] [EC:2.3.2.23]
131549741  ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
131549779  fbxo2; F-box only protein 2 [KO:K10099]
131549949  yod1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
131550500  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
131550853  mapk8b; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
131551018  sec31b; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
131551123  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
131551129  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
131551218  ube2d1b; ubiquitin-conjugating enzyme E2 D1b [KO:K06689] [EC:2.3.2.23]
131551235  dnajb12b; dnaJ homolog subfamily B member 12b isoform X1 [KO:K09518]
131551682  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
131551846  erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
131551854  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
131551991  prkn; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
131551992  capn1a; calpain 1, (mu/I) large subunit a [KO:K01367] [EC:3.4.22.52]
131551993  capn2a; calpain 2, (m/II) large subunit a [KO:K03853] [EC:3.4.22.53]
131552105  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
131552218  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
131552317  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
131552530  ube2d3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
131552683  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
131552972  si:ch211-274f20.2; calnexin [KO:K08054]
131552979  sil1; nucleotide exchange factor SIL1 [KO:K14001]
131553087  ube2d2l; ubiquitin-conjugating enzyme E2D 2 (UBC4/5 homolog, yeast), like isoform X1 [KO:K06689] [EC:2.3.2.23]
131553183  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
131553337  sec24b; LOW QUALITY PROTEIN: protein transport protein Sec24B [KO:K14007]
131553381  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
131553406  canx; calnexin [KO:K08054]
131553474  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
131553534  wfs1b; wolframin [KO:K14020]
131553772  ubxn1; UBX domain-containing protein 1 [KO:K24348]
131553886  magt1; magnesium transporter protein 1 [KO:K19478]
131553914  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
131554078  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
131554100  hspa8b; LOW QUALITY PROTEIN: heat shock protein family A (Hsp70) member 8b [KO:K03283]
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
omc00510  N-Glycan biosynthesis
omc03050  Proteasome
omc03060  Protein export
omc04210  Apoptosis
KO pathway
ko04141   

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