KEGG   PATHWAY: cvg04141
Entry
cvg04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Cyprinodon variegatus (sheepshead minnow)
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
cvg04141  Protein processing in endoplasmic reticulum
cvg04141

Other DBs
GO: 0030433 0034976
Organism
Cyprinodon variegatus (sheepshead minnow) [GN:cvg]
Gene
107100419  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
107094024  sec61a1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
107101088  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
107093952  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
107094232  sec62; translocation protein SEC62 [KO:K12275]
107097372  sec63; translocation protein SEC63 homolog [KO:K09540]
107095882  translocation protein SEC63 homolog [KO:K09540]
107091506  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1-like [KO:K12666]
107093913  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1-like [KO:K12666]
107093194  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
107098277  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
107094445  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
107086177  stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
107101788  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
107099796  magnesium transporter protein 1-like [KO:K19478]
107094644  tumor suppressor candidate 3 [KO:K19478]
107086395  ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
107096153  tmem258; transmembrane protein 258 [KO:K26502]
107091135  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
107098900  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
107102259  ribosome-binding protein 1-like isoform X1 [KO:K14000]
107100412  sil1; nucleotide exchange factor SIL1 [KO:K14001]
107102693  hyou1; hypoxia up-regulated protein 1 [KO:K09486]
107095989  hspa5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
107104371  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
107104668  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
107095621  dnaJ homolog subfamily C member 3-like [KO:K09523]
107093560  dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
107104550  dnaJ homolog subfamily C member 10-like [KO:K09530] [EC:1.8.4.-]
107086009  hsp90b1; endoplasmin [KO:K09487]
107090989  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
107086906  glucosidase 2 subunit beta-like isoform X1 [KO:K08288]
107083083  prkcsh; glucosidase 2 subunit beta [KO:K08288]
107083799  canx; calnexin [KO:K08054]
107092205  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
107084492  protein disulfide-isomerase A3-like [KO:K08056] [EC:5.3.4.1]
107101296  calreticulin-like [KO:K08057]
107080670  calreticulin-like [KO:K08057]
107083523  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA-like [KO:K01230] [EC:3.2.1.113]
107098218  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
107084733  mannosyl-oligosaccharide 1,2-alpha-mannosidase IC-like [KO:K01230] [EC:3.2.1.113]
107088025  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like [KO:K23741] [EC:3.2.1.209]
107083730  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like [KO:K23741] [EC:3.2.1.209]
107082727  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
107085791  lman1; protein ERGIC-53 [KO:K10080]
107083583  preb; prolactin regulatory element-binding protein [KO:K14003]
107090688  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
107100844  sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
107089212  GTP-binding protein SAR1b-like [KO:K07953] [EC:3.6.5.-]
107100242  sec13; protein SEC13 homolog [KO:K14004]
107102180  sec31a; protein transport protein Sec31A [KO:K14005]
107100898  sec31b; protein transport protein Sec31B isoform X1 [KO:K14005]
107084887  sec23a; protein transport protein Sec23A [KO:K14006]
107089099  protein transport protein Sec23B [KO:K14006]
107082266  protein transport protein Sec24B-like isoform X1 [KO:K14007]
107088943  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
107102612  protein transport protein Sec24D-like [KO:K14007]
107090691  sec24a; protein transport protein Sec24A [KO:K14007]
107099201  sec24d; protein transport protein Sec24D isoform X1 [KO:K14007]
107096182  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
107093559  UDP-glucose:glycoprotein glucosyltransferase 2-like isoform X1 [KO:K11718] [EC:2.4.1.-]
107097506  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
107105183  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
107088711  ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
107089728  ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
107085503  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
107092256  pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
107081649  pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
107098546  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
107083860  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
107104622  ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
107082138  os9; protein OS-9 [KO:K10088]
107089283  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
107097898  translocon-associated protein subunit alpha-like [KO:K13249]
107098893  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
107099689  ssr2; translocon-associated protein subunit beta [KO:K13250]
107086955  ssr3; translocon-associated protein subunit gamma [KO:K13251]
107086753  ssr4; translocon-associated protein subunit delta [KO:K04571]
107090253  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
107093227  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
107086337  derl1; derlin-1 [KO:K11519]
107103646  derlin-2-like [KO:K13989]
107093934  derlin-2 [KO:K13989]
107102802  ubxn4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
107090974  ubxn1; UBX domain-containing protein 1 [KO:K24348]
107097268  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
107099580  UBX domain-containing protein 6-like [KO:K14011]
107095280  ubxn6; UBX domain-containing protein 6 [KO:K14011]
107104692  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
107096888  svip; small VCP/p97-interacting protein [KO:K14014]
107083680  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
107094393  transitional endoplasmic reticulum ATPase-like [KO:K13525]
107086268  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
107089655  ufd1l; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
107100718  heat shock cognate 71 kDa protein-like [KO:K03283]
107102697  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
107083771  heat shock cognate 71 kDa protein-like [KO:K03283]
107087461  heat shock 70 kDa protein [KO:K03283]
107097702  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
107090560  dnaJ homolog subfamily A member 2-like [KO:K09503]
107085060  dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
107094392  dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
107093392  dnaJ homolog subfamily B member 1-like [KO:K09507]
107088036  dnaJ homolog subfamily B member 6-like isoform X1 [KO:K09508]
107100328  dnaJ homolog subfamily B member 12-like [KO:K09518]
107100893  dnajb12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
107103370  dnaJ homolog subfamily C member 5-like [KO:K09525]
107080869  dnaJ homolog subfamily C member 5-like [KO:K09525]
107090698  dnaJ homolog subfamily C member 5-like [KO:K09525]
107091578  dnaJ homolog subfamily C member 5-like [KO:K09525]
107083499  dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
107100299  heat shock protein HSP 90-alpha 1 [KO:K04079]
107100300  heat shock protein HSP 90-alpha [KO:K04079]
107081102  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
107099105  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
107098296  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
107084583  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
107095118  hspbp1; hsp70-binding protein 1 [KO:K09562]
107104861  cryaa; alpha-crystallin A chain [KO:K09541]
107089805  alpha-crystallin B chain-like [KO:K09542]
107086141  hspb6; heat shock protein beta-6 [KO:K09542]
107085162  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
107084124  plaa; phospholipase A-2-activating protein [KO:K14018]
107100424  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
107098148  rad23a; UV excision repair protein RAD23 homolog A [KO:K10839]
107083285  ubqln4; ubiquilin-4 [KO:K04523]
107084438  ubqln1; ubiquilin-1 [KO:K04523]
107104455  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
107099126  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
107086900  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
107102124  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
107080830  eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
107082057  nuclear factor erythroid 2-related factor 2-like [KO:K05638]
107091869  nfe2; transcription factor NF-E2 45 kDa subunit [KO:K05638]
107085767  nuclear factor erythroid 2-related factor 2-like [KO:K05638]
107087200  atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
107090074  cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
107102812  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
107080667  apoptosis regulator Bcl-2-like [KO:K02161]
107083382  apoptosis regulator Bcl-2-like [KO:K02161]
107098581  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
107104477  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
107085724  wolframin-like isoform X1 [KO:K14020]
107096697  wolframin-like [KO:K14020]
107101009  wolframin-like [KO:K14020]
107082488  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
107081826  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
107091970  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
107086406  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107081787  mitogen-activated protein kinase mpkC-like isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107092832  ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107092860  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
107096169  traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
107103870  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
107084615  mitogen-activated protein kinase kinase kinase 5-like [KO:K04426] [EC:2.7.11.25]
107082864  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
107101318  map2k7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
107085828  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
107101533  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
107102050  mitogen-activated protein kinase 9-like isoform X1 [KO:K04440] [EC:2.7.11.24]
107086602  mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
107081311  apoptosis regulator BAX-like [KO:K02159]
107084722  apoptosis regulator BAX-like [KO:K02159]
107086239  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
107102261  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
107082646  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
107103873  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
107102256  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
107102257  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
107082647  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
107082675  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
107091686  E3 ubiquitin-protein ligase MARCH6-like isoform X1 [KO:K10661] [EC:2.3.2.27]
107096090  march6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
107101718  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
107099521  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
107098740  ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
107104226  ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
107095546  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
107090166  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
107086185  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
107091300  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
107087019  vimp; selenoprotein S isoform X1 [KO:K14025]
107088484  sel1l; protein sel-1 homolog 1 [KO:K14026]
107096277  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
107082783  amfr; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
107085028  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
107082307  ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
107096805  ubiquitin-conjugating enzyme E2 D2-like isoform X1 [KO:K06689] [EC:2.3.2.23]
107083810  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
107082391  ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
107084667  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
107101386  park2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
107093096  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
107097881  cul1; cullin-1 [KO:K03347]
107085893  skp1; S-phase kinase-associated protein 1 [KO:K03094]
107097888  F-box only protein 2-like [KO:K10099]
107083932  F-box only protein 6-like [KO:K10100]
107083892  F-box only protein 44-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
cvg00510  N-Glycan biosynthesis
cvg03050  Proteasome
cvg03060  Protein export
cvg04210  Apoptosis
KO pathway
ko04141   
LinkDB

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