KEGG   PATHWAY: pgut04141
Entry
pgut04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Pantherophis guttatus (corn snake)
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
pgut04141  Protein processing in endoplasmic reticulum
pgut04141

Other DBs
GO: 0030433 0034976
Organism
Pantherophis guttatus (corn snake) [GN:pgut]
Gene
117668306  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
117673853  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
117664926  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
117664225  protein transport protein Sec61 subunit gamma-like [KO:K07342]
117664982  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
117678204  SEC62; translocation protein SEC62 [KO:K12275]
117660826  SEC63; translocation protein SEC63 homolog [KO:K09540]
117673844  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
117663628  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
117658775  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
117656557  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
117668193  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
117656010  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
132710171  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
117676027  MAGT1; magnesium transporter protein 1 [KO:K19478]
117654588  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
117679927  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
117667032  TMEM258; transmembrane protein 258 isoform X1 [KO:K26502]
117670708  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
117655841  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
117674323  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
117670201  hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
117658399  HSPA5; LOW QUALITY PROTEIN: endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
117678475  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
117655090  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
117665579  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
117679695  dnaJ homolog subfamily C member 3-like [KO:K09523]
117672275  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
117655386  HSP90B1; endoplasmin [KO:K09487]
117675640  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
117655398  CANX; calnexin [KO:K08054]
117676233  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
117664222  calreticulin-like [KO:K08057]
117675626  CALR; calreticulin [KO:K08057]
117677401  CALR3; calreticulin-3 [KO:K08057]
117661840  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
117666222  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
117663380  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
117676613  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
117679715  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
117680016  LMAN1; protein ERGIC-53 [KO:K10080]
117667635  PREB; prolactin regulatory element-binding protein [KO:K14003]
117655998  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
117670668  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
117673905  SEC13; protein SEC13 homolog [KO:K14004]
117673673  SEC31A; protein transport protein Sec31A isoform X2 [KO:K14005]
117665797  SEC23A; protein transport protein Sec23A [KO:K14006]
117662343  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
117655948  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
117676096  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
117679932  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
117680202  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
117665571  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
117678551  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
117673922  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
117663692  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
117661107  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
117663040  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
117664966  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
117674228  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
117672258  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
132709059  endoplasmic reticulum resident protein 29-like [KO:K09586]
117672468  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
117675066  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
117671965  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
117666502  OS9; protein OS-9 isoform X1 [KO:K10088]
117671514  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
117672461  SSR1; translocon-associated protein subunit alpha [KO:K13249]
117671344  SSR2; LOW QUALITY PROTEIN: translocon-associated protein subunit beta [KO:K13250]
117678253  SSR3; translocon-associated protein subunit gamma [KO:K13251]
117675503  SSR4; translocon-associated protein subunit delta [KO:K04571]
117675492  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
117663588  translocating chain-associated membrane protein 1-like 1 isoform X1 [KO:K14010]
117672877  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
117668391  DERL1; derlin-1 [KO:K11519]
117667496  DERL2; derlin-2 [KO:K13989]
117660001  UBX domain-containing protein 1 [KO:K24348]
117669668  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
132709962  UBXN1; UBX domain-containing protein 1 [KO:K24348]
117674285  UBXN2A; LOW QUALITY PROTEIN: UBX domain-containing protein 2A [KO:K24349]
117668579  UBXN6; LOW QUALITY PROTEIN: UBX domain-containing protein 6 [KO:K14011]
117665410  SVIP; small VCP/p97-interacting protein [KO:K14014]
117659287  transitional endoplasmic reticulum ATPase-like [KO:K13525]
117679742  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
117662970  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
117676356  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
117670081  heat shock 70 kDa protein 1-like [KO:K03283]
117670082  heat shock 70 kDa protein II-like [KO:K03283]
117673232  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
117677630  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283]
117661720  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
117670102  dnaJ homolog subfamily A member 1-like [KO:K09502]
117670920  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
117659339  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
117675286  dnaJ homolog subfamily B member 6-like [KO:K09508]
117675288  dnaJ homolog subfamily B member 6-like isoform X1 [KO:K09508]
117675291  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
117670662  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
117654853  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
117667628  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
117672849  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
117665996  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
117671840  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
117661850  HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485]
117680153  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
117664910  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
117676886  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
117669091  HSPBP1; hsp70-binding protein 1 [KO:K09562]
117669450  CRYAA; alpha-crystallin A chain [KO:K09541]
117669310  HSPB6; heat shock protein beta-6 [KO:K09542]
117670249  CRYAB; alpha-crystallin B chain [KO:K09542]
117663856  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
117661549  PLAA; phospholipase A-2-activating protein [KO:K14018]
117661614  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
117675627  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
117661404  UBQLN1; ubiquilin-1 [KO:K04523]
117676457  ubiquilin-1-like [KO:K04523]
117676458  ubiquilin-1-like [KO:K04523]
117676460  ubiquilin-1-like [KO:K04523]
117671206  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
117656038  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
117665922  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
117678119  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
117664790  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
117665555  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
117671941  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
117655911  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117666519  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
117674759  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
117661204  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
117667838  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
117669958  wolframin isoform X1 [KO:K14020]
132712637  wolframin-like [KO:K14020]
117670395  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
117675343  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
117672317  LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
132711953  LOW QUALITY PROTEIN: X-box-binding protein 1-like [KO:K09027]
117662897  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117667385  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117667903  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
117676633  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
117666295  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
117658997  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
117664553  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117655531  mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
117678928  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
132710426  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117669122  apoptosis regulator BAX-like isoform X1 [KO:K02159]
117669123  apoptosis regulator BAX-like isoform X1 [KO:K02159]
117663987  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
117669752  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
117671847  CAPN11; calpain-11 [KO:K01367] [EC:3.4.22.52]
117671750  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
117662438  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
117660766  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
117675858  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
117678486  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
117669754  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
117667825  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
117677917  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
117669866  SELENOS; selenoprotein S [KO:K14025]
117665886  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
117674414  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
132712235  protein sel-1 homolog 1-like isoform X1 [KO:K14026]
117670401  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
117670322  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
117667254  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
117662334  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
117670651  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
117679892  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
117672071  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
117655425  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
117664970  CUL1; cullin-1 [KO:K03347]
117656074  SKP1; S-phase kinase-associated protein 1 isoform X1 [KO:K03094]
117659696  FBXO2; F-box only protein 2 [KO:K10099]
117659695  F-box only protein 6-like isoform X2 [KO:K10100]
117673066  F-box only protein 44-like [KO:K10100]
117673067  F-box only protein 44-like [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
pgut00510  N-Glycan biosynthesis
pgut03050  Proteasome
pgut03060  Protein export
pgut04210  Apoptosis
KO pathway
ko04141   
LinkDB

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