KEGG   PATHWAY: hhip04141
Entry
hhip04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Hippoglossus hippoglossus (Atlantic halibut)
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
hhip04141  Protein processing in endoplasmic reticulum
hhip04141

Other DBs
GO: 0030433 0034976
Organism
Hippoglossus hippoglossus (Atlantic halibut) [GN:hhip]
Gene
117751779  sec24d; protein transport protein Sec24D isoform X1 [KO:K14007]
117751968  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
117752243  plaa; phospholipase A-2-activating protein [KO:K14018]
117752270  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
117752542  mapk8b; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117752599  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
117752862  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
117752901  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
117752907  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
117752992  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
117753016  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
117753209  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
117753256  ube2d1b; ubiquitin-conjugating enzyme E2 D1b [KO:K06689] [EC:2.3.2.23]
117753552  [KO:K03347]
117753579  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
117753795  pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
117753892  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
117753923  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
117753974  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
117753995  [KO:K10661] [EC:2.3.2.27]
117754199  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
117754432  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
117754484  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
117754557  ubxn4; UBX domain-containing protein 4 [KO:K24348]
117754696  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
117754769  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
117755227  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
117755254  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
117755255  dnajc3a; dnaJ homolog subfamily C member 3a isoform X1 [KO:K09523]
117755477  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
117755702  [KO:K09525]
117755714  atxn3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
117755731  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
117755974  sec23a; protein transport protein Sec23A isoform X1 [KO:K14006]
117756127  [KO:K01367] [EC:3.4.22.52]
117756184  [KO:K01367] [EC:3.4.22.52]
117756185  [KO:K01367] [EC:3.4.22.52]
117756336  [KO:K06689] [EC:2.3.2.23]
117756367  hspa4l; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
117756372  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
117757544  ckap4; cytoskeleton-associated protein 4 isoform X1 [KO:K13999]
117757967  pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
117758005  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
117758059  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
117758073  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
117758074  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
117758147  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
117758196  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
117758216  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
117758218  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
117758302  map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
117758314  [KO:K10636] [EC:2.3.2.36]
117758797  sec63; translocation protein SEC63 homolog [KO:K09540]
117758855  sel1l; protein sel-1 homolog 1 [KO:K14026]
117759343  [KO:K06689] [EC:2.3.2.23]
117759410  atf4a; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117759448  zgc:153993; apoptosis regulator BAX [KO:K02159]
117759527  selenos; selenoprotein S isoform X1 [KO:K14025]
117759776  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
117760000  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
117760739  calr; calreticulin [KO:K08057]
117760767  rad23ab; RAD23 homolog A, nucleotide excision repair protein b isoform X1 [KO:K10839]
117760769  [KO:K08057]
117760838  ubxn6; UBX domain-containing protein 6 [KO:K14011]
117760944  ddit3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
117761017  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
117761104  yod1; LOW QUALITY PROTEIN: ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
117761212  [KO:K13249]
117761222  wfs1a; wolframin isoform X1 [KO:K14020]
117761298  os9; protein OS-9 isoform X1 [KO:K10088]
117761362  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
117761372  [KO:K10956]
117761469  [KO:K10956]
117761565  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
117761570  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
117761889  fbxo2; F-box only protein 2 [KO:K10099]
117762220  sec13; protein SEC13 homolog [KO:K14004]
117762324  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
117762620  svip; small VCP/p97-interacting protein isoform X1 [KO:K14014]
117762701  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
117762904  tmem258; transmembrane protein 258 [KO:K26502]
117762973  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
117762984  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
117763557  [KO:K28771]
117763662  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
117763787  hsp90b1; LOW QUALITY PROTEIN: endoplasmin [KO:K09487]
117763829  amfra; autocrine motility factor receptor a isoform X1 [KO:K10636] [EC:2.3.2.36]
117763874  [KO:K01367] [EC:3.4.22.52]
117763891  capn2l; calpain 2, (m/II) large subunit, like [KO:K03853] [EC:3.4.22.53]
117763907  capn8; calpain-8 [KO:K03853] [EC:3.4.22.53]
117763923  [KO:K03853] [EC:3.4.22.53]
117764081  ero1b; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
117764146  zgc:113278; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
117764238  [KO:K09525]
117764311  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
117764324  nfe2; transcription factor NF-E2 45 kDa subunit [KO:K05638]
117764428  [KO:K10100]
117764574  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
117764760  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
117764777  ssr4; translocon-associated protein subunit delta [KO:K04571]
117764952  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
117765067  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
117765069  tusc3; tumor suppressor candidate 3 [KO:K19478]
117765258  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
117765414  [KO:K03283]
117765415  [KO:K03283]
117765644  sec23b; protein transport protein Sec23B [KO:K14006]
117765697  dnajb1a; dnaJ homolog subfamily B member 1a isoform X1 [KO:K09507]
117765997  [KO:K09561] [EC:2.3.2.27]
117766292  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
117766362  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
117766567  atf4b; activating transcription factor 4b [KO:K04374]
117766590  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117766633  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
117766753  hspbp1; hsp70-binding protein 1 [KO:K09562]
117766791  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
117766953  [KO:K02159]
117766958  prkcsh; glucosidase 2 subunit beta [KO:K08288]
117767233  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
117767372  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117767786  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
117767827  man1b1b; mannosidase, alpha, class 1B, member 1b isoform X1 [KO:K23741]
117767837  traf2a; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
117768006  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
117768123  [KO:K04523]
117768200  [KO:K10575] [EC:2.3.2.23]
117768320  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
117768331  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
117768480  derl3; derlin-3 [KO:K13989]
117768512  si:dkey-13n15.2; protein transport protein Sec24C [KO:K14007]
117768646  zgc:136908; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
117769022  dnajb1b; dnaJ homolog subfamily B member 1b [KO:K09507]
117769095  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
117769162  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
117769194  [KO:K06689] [EC:2.3.2.23]
117769197  ubxn1; UBX domain-containing protein 1 [KO:K24348]
117769257  mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
117769325  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
117769474  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
117769530  [KO:K14020]
117769696  sil1; nucleotide exchange factor SIL1 [KO:K14001]
117769703  [KO:K19478]
117769727  canx; calnexin [KO:K08054]
117769876  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
117770099  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
117770112  stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
117770392  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
117770623  [KO:K04523]
117770649  atf6b; LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
117770802  pdia8; protein disulfide isomerase family A, member 8 [KO:K08056] [EC:5.3.4.1]
117771140  ssr2; translocon-associated protein subunit beta [KO:K13250]
117771243  traf2b; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
117771259  [KO:K03173] [EC:2.3.2.27]
117771541  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:5.6.1.10]
117771580  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
117771596  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
117771648  [KO:K10575] [EC:2.3.2.23]
117771676  [KO:K03283]
117771942  derl2; derlin-2 isoform X1 [KO:K13989]
117771995  lman1; protein ERGIC-53 [KO:K10080]
117772012  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
117772179  rnf185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
117772227  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
117772246  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
117772329  cryaa; alpha-crystallin A chain [KO:K09541]
117772425  ssr3; translocon-associated protein subunit gamma [KO:K13251]
117774110  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
117774132  [KO:K03283]
117774133  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
117774134  [KO:K03283]
117774290  skp1; S-phase kinase-associated protein 1 [KO:K03094]
117774453  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
117774457  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
117774599  [KO:K09542]
117774803  [KO:K01367] [EC:3.4.22.52]
117774959  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
117775066  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
117775419  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
117775447  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
117775533  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
117775547  [KO:K06689] [EC:2.3.2.23]
117775632  [KO:K03853] [EC:3.4.22.53]
117775686  prkn; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
117775780  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117775857  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
117776037  [KO:K03853] [EC:3.4.22.53]
117776079  [KO:K01367] [EC:3.4.22.52]
117776080  [KO:K03853] [EC:3.4.22.53]
117776320  [KO:K04523]
117776497  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
117776557  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
117776567  hspb6; heat shock protein beta-6 isoform X1 [KO:K09542]
117776916  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
117777343  preb; prolactin regulatory element-binding protein [KO:K14003]
117777385  derl1; derlin-1 [KO:K11519]
117777402  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
117777415  [KO:K01230] [EC:3.2.1.113]
117777844  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
117777864  [KO:K09525]
117778179  [KO:K10661] [EC:2.3.2.27]
117778191  calr3b; calreticulin 3b [KO:K08057]
117778280  sec62; translocation protein SEC62 isoform X1 [KO:K12275]
117778378  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
117778459  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
117778944  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
117779078  [KO:K08288]
117779098  [KO:K01367] [EC:3.4.22.52]
117779099  [KO:K01367] [EC:3.4.22.52]
117779391  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
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
hhip00510  N-Glycan biosynthesis
hhip03050  Proteasome
hhip03060  Protein export
hhip04210  Apoptosis
KO pathway
ko04141   

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